Long-term aesthetics, patient-reported outcomes, and auricular sensitivity after microtia reconstruction: A systematic review.

BACKGROUND: Auricular reconstruction for microtia is most frequently performed using autologous costal cartilage (ACC) or porous polyethylene (PPE) implants. Short-term results are generally promising, but long-term results remain unclear. Long-term outcomes were explored in this systematic review, and minimal reporting criteria were suggested for future original data studies. METHODS: A systematic literature search was conducted in MEDLINE, EMBASE, and the Cochrane Central Register of Controlled Trials from inception through October 14, 2020. Articles on auricular reconstruction in patients with microtia using ACC or PPE were included if postsurgical follow-up was at least 1 year. Outcome reporting was split into separate publications, and results on complications were reported previously. This publication focused on long-term aesthetic, patient-reported, and sensitivity outcomes. RESULTS: Forty-one publications reported on these outcomes. Both materials led to aesthetically pleasing results and high rates of patient satisfaction. ACC frameworks grew similarly to contralateral ears, and the anterior surface of auricles regained sensitivity. Furthermore, postoperative health-related quality of life (HRQoL) outcomes were generally good. Data synthesis was limited due to considerable variability between studies and poor study quality. No conclusions could be drawn on the superiority of either method due to the lack of comparative analyses. CONCLUSION: Future studies should minimally report (1) surgical efficacy measured using the tool provided in the UK Care Standards for the Management of Patients with Microtia and Atresia; (2) complications including framework extrusion or exposure, graft loss, framework resorption, wire exposure and scalp/auricular scar complications and (3) HRQoL before and after treatment using the EAR-Q patient-reported outcome measure (PROM).

Long-term complications of microtia reconstruction: A systematic review.

BACKGROUND: Microtia is a rare disorder characterized by malformation or even complete absence of the auricle. Reconstruction is often performed using autologous costal cartilage (ACC) or porous polyethylene implants (PPE). However, the long-term outcomes of both methods are unclear. OBJECTIVE: This systematic review aimed to analyze long-term complications and suggest minimal reporting criteria for future original data studies. METHODS: A systematic literature search was conducted in MEDLINE, EMBASE and the Cochrane Central Register of Controlled Trials from inception through October 14, 2020. Articles on auricular reconstruction in patients with microtia using ACC or PPE were included provided that the follow-up period was at least one year. This publication focused on long-term complications reported in patients with a postoperative follow-up period of at least one year. RESULTS: Twenty-nine publications reported on complications during long-term follow-up. Overall long-term complication rates were not reported. The incidence of individual complications during long-term follow-up was less than 10% after ACC reconstruction and less than 15% in PPE reconstruction. Framework resorption and wire exposure were reported even after an extended follow-up of more than five years after ACC reconstruction, while reports on the extended long-term results of PPE reconstruction are limited. Data synthesis was limited due to heterogeneity and poor study quality. CONCLUSIONS: Future studies should report on long-term complications including framework exposure or extrusion, graft loss, framework resorption, wire exposure and scalp and auricular scar complications. We recommend a surgical follow-up of at least five years.

Biofabrication of a shape-stable auricular structure for the reconstruction of ear deformities.

Bioengineering of the human auricle remains a significant challenge, where the complex and unique shape, the generation of high-quality neocartilage, and shape preservation are key factors. Future regenerative medicine-based approaches for auricular cartilage reconstruction will benefit from a smart combination of various strategies. Our approach to fabrication of an ear-shaped construct uses hybrid bioprinting techniques, a recently identified progenitor cell population, previously validated biomaterials, and a smart scaffold design. Specifically, we generated a 3D-printed polycaprolactone (PCL) scaffold via fused deposition modeling, photocrosslinked a human auricular cartilage progenitor cell-laden gelatin methacryloyl (gelMA) hydrogel within the scaffold, and cultured the bioengineered structure in vitro in chondrogenic media for 30 days. Our results show that the fabrication process maintains the viability and chondrogenic phenotype of the cells, that the compressive properties of the combined PCL and gelMA hybrid auricular constructs are similar to native auricular cartilage, and that biofabricated hybrid auricular structures exhibit excellent shape fidelity compared with the 3D digital model along with deposition of cartilage-like matrix in both peripheral and central areas of the auricular structure. Our strategy affords an anatomically enhanced auricular structure with appropriate mechanical properties, ensures adequate preservation of the auricular shape during a dynamic in vitro culture period, and enables chondrogenically potent progenitor cells to produce abundant cartilage-like matrix throughout the auricular construct. The combination of smart scaffold design with 3D bioprinting and cartilage progenitor cells holds promise for the development of clinically translatable regenerative medicine strategies for auricular reconstruction.

Microtia and cholesteatoma: Implications for the reconstructive surgeon.

Infection after reconstructive surgery for microtia is a technical challenge. This can be a sign of cholesteatoma formation by entrapment of epithelium in the middle or outer ear, specifically when the patient does not respond to first choice antibiotic therapy and debridement. Two patients with microtia presented themselves with severe infections after ear reconstruction. In both cases cholesteatoma was diagnosed as the cause of the infection. After cholesteatoma management an additional surgical procedure was necessary to improve the esthetic outcome. The plastic surgeon should identify possible signs of cholesteatoma after reconstruction of the auricle.

Surgical management in submucous cleft palate patients.

OBJECTIVES: The submucous cleft palate (SMCP) is considered to be the most subtle type of cleft palate. Early detection is important to allow on time intervention by speech therapy and/or surgical repair before the children already develop compensatory speech mechanisms. The purpose of this study was to investigate at what time children with a SMCP present, to determine when children are operated, and to analyze the postoperative outcomes for in SMCP children. PATIENT AND METHODS: Medical records from 766 individuals registered in the cleft registry in the Wilhelmina's Children's' Hospital, Utrecht, were retrospectively reviewed. Inclusion criteria were children diagnosed with SMCP. The following data were collected: age at diagnosis, physical examination, age at surgery, surgical technique, speech therapy pre- and post-surgery, otitis media, secondary cleft surgery, family history, syndromes, and other anomalies. RESULTS: In total, 56 SMCP children were identified. The mean age of diagnosis was 44.0 months (range 0-150, SD = 37.0). In 48 children (85.7%), surgical intervention was performed (Furlow plasty, intravelar veloplasty, pharyngoplasty, or Furlow combined with buccal flap). CONCLUSION: This retrospective study reconfirms that SMCP often presents late, even in a country with a modern healthcare system and adequate follow-up of all newborns by the so-called youth doctors in "children's healthcare centers" up to the age of 4 years old. Almost 86% of patients ultimately needed palate surgery when SMCP was suspected. CLINICAL RELEVANCE: Any child presenting with repeated episodes of otitis media, nasal regurgitation, or speech difficulties should have prompt consideration for SMCP as diagnosis.

Hearing-related quality of life, developmental outcomes and performance in children and young adults with unilateral conductive hearing loss due to aural atresia.

BACKGROUND: While research has shown that children with single sided deafness have a lower quality of life and developmental outcomes compared to normal hearing peers, little is known about these domains in children with unilateral congenital conductive hearing loss due to aural atresia. OBJECTIVES: This study aims to investigate the hearing-related quality of life, developmental outcomes and educational performance in children and young adults with unilateral conductive hearing loss due to aural atresia. METHODS: Nineteen children and young adults with unilateral aural atresia received a set of five questionnaires. Hearing-related quality of life (SSQ), general quality of life (Kidscreen-27), speech and language development (CCC-2-Nl), educational performance and problems in social-emotional and behavioral domains (CBCL/YSR/ASR) were measured with validated questionnaires. Scores on the questionnaires were compared to their norm scores. Mann-Whitney U tests and independent t-tests were used to identify significant differences between age groups. RESULTS: Mean scores on the SSQ subscales were speech 6.78, spatial 5.00 and quality 6.98. Mean scores on the Kidscreen-27, CCC-2-NL, CBCL/YSR/ASR fell within normal or non-clinical range. A high number of cases needed speech therapy (60.7%) or special measures in class (79.3%) or showed grade repetition (>30%) in primary or secondary school. CONCLUSION: Children and young adults with unilateral conductive hearing loss due to congenital aural atresia showed lower scores regarding hearing-related quality of life compared to normal-hearing peers. The result show similarities with children with single sided deafness. Regarding general quality of life, speech and language development and in social-emotional and behavioral domains the studied children and young adults seem to develop according to norm scores. It is important to observe these children closely as they may need guidance during education to allow them to thrive.

Incidence of cleft-related speech problems in children with an isolated cleft lip.

OBJECTIVES: Clinicians agree that children with isolated cleft lip have fewer cleft-associated problems than children with cleft lip and palate. Unfortunately, for isolated cleft lip children, the risk of cleft-associated problems is unknown and maybe underestimated. Often, these children do not get the required follow-up by a multidisciplinary team and thereby not the known benefits in supporting their development. This study examines the incidence of cleft-related speech problems and ear problems in children with isolated cleft lip. MATERIALS AND METHODS: A prospective study was performed on all children born with an isolated cleft lip and treated at the Wilhelmina Children's Hospital in Utrecht between January 2007 and April 2014. Data were collected for sex, date of birth, genetics, cleft lip type, date of cleft lip repair, type of repair, speech/language problems, and ear problems. RESULTS: This study included 75 patients (59% male). The mean age of the children at the moment of speech examination was 32.5 months (SD 6.1). Eighteen of the 75 children (24%) needed speech and language therapy; however, only one child (1.3%) had a cleft-related speech problem. Sixteen of the 75 patients (21%) reported a history of one or more episodes of acute otitis media (AOM)/otitis media with effusion (OME) during the first 6 years. CONCLUSION/CLINICAL RELEVANCE: This is the first prospective study analyzing the incidence of cleft-related speech problems in children with an isolated cleft lip. These children do not have a higher risk of cleft-related speech problems or AOM/OME when compared to the general population. However, children with an isolated cleft do have a higher incidence of speech therapy.

Effect of hyperextension of the neck (rose position) on cerebral blood oxygenation in patients who underwent cleft palate reconstructive surgery: prospective cohort study using near-infrared spectroscopy.

OBJECTIVES: To facilitate the best approach during cleft palate surgery, children are positioned with hyperextension of the neck. Extensive head extension may induce intraoperative cerebral ischemia if collateral flow is insufficient. To evaluate and monitor the effect of cerebral blood flow on cerebral tissue oxygenation, near-infrared spectroscopy has proved to be a valuable method. The aim of this study was to evaluate and quantify whether hyperextension affects the cerebral tissue oxygenation in children during cleft palate surgery. MATERIALS AND METHODS: This prospective study included children (ASA 1 and 2) under the age of 3 years old who underwent cleft palate repair at the Wilhelmina Children's Hospital, in The Netherlands. Data were collected for date of birth, cleft type, date of cleft repair, and physiological parameters (MAP, saturation, heart rate, expiratory CO2 and O2, temperature, and cerebral blood oxygenation) during surgery. The cerebral blood oxygenation was measured with NIRS. RESULTS: Thirty-four children were included in this study. The majority of the population was male (61.8%, n = 21). The mixed model analyses showed a significant drop at time of Rose position of - 4.25 (69-74 95% CI; p < 0.001) and - 4.39 (69-74 95% CI; p < 0.001). Postoperatively, none of the children displayed any neurological disturbance. CONCLUSION: This study suggests that hyperextension of the head during cleft palate surgery leads to a significant decrease in cerebral oxygenation. Severe cerebral desaturation events during surgery were uncommon and do not seem to be of clinical relevance in ASA 1 and 2 children. CLINICAL RELEVANCE: There was a significant drop in cerebral oxygenation after positioning however it is not clear whether this drop is truly significant physiologically in ASA 1 and 2 patients.

If it ain't broke don't fix it? Ethics of splinting deformed newborn ears.

Neonatal ear splinting is a proven and safe method to mold deformed ears into a more common shape. Based on our earlier studies, splinting is recommended only before the age of six weeks and preferably within the first week after birth. This can be done by initiating a system in which this intervention is actively proposed to parents. In this paper, we ethically evaluate such a system. By molding perfectly healthy newborn ears, we reach the boundary between treatment and enhancement. A key question is, therefore, whether we could classify neonatal ear splinting as a therapy. On the level of the individual, the advantages outweigh the drawbacks, but on the level of society, it is more complicated. Making ear deformities a part of official national screening programs fails to meet WHO criteria. Moreover, by systematically offering ear molding, professionals may be promoting guilt or fear of missing the opportunity. Additionally, it could affect societal attitudes toward cosmetic deformities. However, if we argue that on the individual level infants may benefit from ear splinting, then active detection of ear deformities allows parents to choose in a timely way from the full range of options, including splinting and a wait-and-see approach. We are inclined to optimally inform parents without setting up a full-blown public health program. The extent to which it is possible to timely offer splints to parents of newborns depends on the infrastructure of health care systems. The key will be for everyone involved, public or commercial, to responsibly educate and facilitate.

Impact of unilateral congenital aural atresia on academic Performance: A systematic review.

BACKGROUND: Little is known about the academic performance of children with unilateral congenital aural atresia (CAA). OBJECTIVE: of review: Our objective was to summarize what is known about the academic performance of children with hearing loss by unilateral congenital aural atresia, in order to provide pragmatic recommendations to clinicians who see children with this entity. TYPE OF REVIEW: Systematic review. SEARCH STRATEGY: We conducted a systematic search in PubMed Medline, EMBASE, and Cochrane Library combining the terms "atresia" and synonyms with "unilateral hearing loss" and synonyms. Date of the most recent search was 16 May 2018. EVALUATION METHOD: Two independent authors identified studies, extracted data, and assessed risk of bias. This review was reported according to the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA). Observational studies on the academic achievements of patients of any age with unilateral conductive hearing loss of any level due to congenital aural atresia were included. We considered grade retention, special education, individualized education plans, and parental report of school performance as outcome measures for academic achievement. RESULTS: Two studies reporting on academic performance of patients with unilateral CAA, which both had a significant risk of bias. One study (n = 140) showed a grade retention rate of 3.6% (n = 5) in total. 15.7% (n = 22) needed special education, and 36.4% (n = 51) used an individualized education program. The second study, reporting on 67 patients with unilateral CAA, showed that 29.9% (n = 20) of the patients received school intervention, and 25.4% (n = 17) had learning problems. CONCLUSION: Current evidence regarding the effect of unilateral congenital aural atresia on academic performance is sparse, inconclusive and has a significant risk of bias. High quality observational studies assessing the effects of aural atresia on academic performance in these patients should be initiated.

Abnormal soft palate movements in patients with microtia.

INTRODUCTION: Microtia is a congenital malformation of the auricle, ranging in severity. It can be isolated or associated with (craniofacial) anomalies. Most of these anomalies together are described as being part of the oculo-auriculo-vertebral spectrum (OAVS). Velar abnormalities have been described to occur in patients with OAVS; however, the incidence in patients with microtia without OAVS is largely unknown. The primary purpose of this study was to examine the prevalence of velar palsy in patients with isolated microtia and patients with microtia and signs of OAVS. The secondary purpose was to identify possible risk factors associated with the presence of velar palsy. METHODS: All patients with microtia presented to our department between January 2015 and March 2017 were retrospectively reviewed. Medical images, information of the palate, and demographic data were extracted from the patients' medical files. RESULTS: Velar palsy was present in 18 out of 42 patients with isolated microtia (43%; no signs of OAVS) and in 33 out of 41 patients with microtia and signs of OAVS (80%). Patients with signs of OAVS were found to be independently associated with a higher prevalence of velar palsy (OR: 4.8; 95% CI: 1.7-13). CONCLUSION: This study demonstrates a clear relationship between abnormal velar movement and microtia. We believe that isolated microtia should not be seen as a separate entity but as a part of OAVS. Abnormal velar movement can lead to velopharyngeal insufficiency (VPI), which can affect speech development in children. Physicians should examine the palate in all patients with microtia and not restrict to those with speech difficulties.

Internationally adopted children with cleft lip and/or cleft palate: Middle ear findings and hearing during childhood.

OBJECTIVE: Adopted children with cleft lip and/or cleft palate form a diverse group of patients. Due to increased age at palatal repair, adopted children have a higher risk of velopharyngeal insuffiency and poor speech outcome. Delayed palate repair may also lead to longer lasting Eustachian tube dysfunction. Decreased function of the Eustachian tube causes otitis media with effusion and recurrent acute otitis media, which can lead to other middle ear problems and hearing loss. METHODS: One-hundred-and-thirty-two adopted children treated by the Cleft palate team in Wilhelmina Children's Hospital during January 1994 and December 2014 were included. Retrospectively, middle ear findings, the need for ventilation tube insertion and hearing during childhood were assessed. Findings were compared with 132 locally born children with cleft lip and/or cleft palate. RESULTS: Adopted children had a mean age of 26.5 months old when they arrived in our country. After the age of two the total number of otitis media with effusion episodes and the need for ventilation tube placement did not significantly differ among adopted and non-adopted children. Adopted children had significantly more tympanic membrane perforations. Hearing threshold levels normalized with increasing age. Although within normal range, adopted children showed significantly higher pure tone averages than locally born children when they were eight to ten years old. CONCLUSION: In general, adopted patients with cleft lip and/or cleft palate did not have more middle ear problems or ventilation tubes during childhood. However, theyhave more tympanic membrane perforations.

Progenitor cells in auricular cartilage demonstrate cartilage-forming capacity in 3D hydrogel culture.

Paramount for the generation of auricular structures of clinically-relevant size is the acquisition of a large number of cells maintaining an elastic cartilage phenotype, which is the key in producing a tissue capable of withstanding forces subjected to the auricle. Current regenerative medicine strategies utilize chondrocytes from various locations or mesenchymal stromal cells (MSCs). However, the quality of neo-tissues resulting from these cell types is inadequate due to inefficient chondrogenic differentiation and endochondral ossification, respectively. Recently, a subpopulation of stem/progenitor cells has been identified within the auricular cartilage tissue, with similarities to MSCs in terms of proliferative capacity and cell surface biomarkers, but their potential for tissue engineering has not yet been explored. This study compared the in vitro cartilage-forming ability of equine auricular cartilage progenitor cells (AuCPCs), bone marrow-derived MSCs and auricular chondrocytes in gelatin methacryloyl (gelMA)-based hydrogels over a period of 56 d, by assessing their ability to undergo chondrogenic differentiation. Neocartilage formation was assessed through gene expression profiling, compression testing, biochemical composition and histology. Similar to MSCs and chondrocytes, AuCPCs displayed a marked ability to generate cartilaginous matrix, although, under the applied culture conditions, MSCs outperformed both cartilage-derived cell types in terms of matrix production and mechanical properties. AuCPCs demonstrated upregulated mRNA expression of elastin, low expression of collagen type X and similar levels of proteoglycan production and mechanical properties as compared to chondrocytes. These results underscored the AuCPCs' tissue-specific differentiation potential, making them an interesting cell source for the next generation of elastic cartilage tissue-engineered constructs.

The use of throat packs in pediatric cleft lip/palate surgery: a retrospective study.

OBJECTIVES: Throat packs are commonly used to prevent ingestion or aspiration of blood and other debris during cleft lip/palate surgery. However, dislodgement or (partial) retainment after extubation could have serious consequences. The aim of the present study was to investigate the effect of omitting pharyngeal packing during cleft lip/palate surgery on the incidence of early postoperative complications in children. MATERIALS AND METHODS: A retrospective study was performed on all children who underwent cleft lip/palate surgery at the Wilhelmina Children's Hospital. This study compared the period January 2010 through December 2012 when pharyngeal packing was applied according to local protocol (group A) with the period January 2013 till December 2015 when pharyngeal packing was no longer applied after removal from the protocol (group B). Data were collected for sex, age at operation, cleft lip/palate type, type of repair, lateral incisions, length of hospital stay, and complications in the first 6 weeks after surgery. Early complications included wound dehiscence, postoperative bleeding, infection, fever, upper respiratory tract infection (URTI), and lower respiratory tract infection (LRTI). RESULTS: This study included 489 cleft lip/palate operations (group A n = 246, group B n = 243). A total of 39 (15.9%) early complications were recorded in group A and a total of 40 (16.5%) in group B. There were no significant differences (P = 0.902) in complications between the two groups; however, there was a significant difference (P < 0.001) in length of hospital stay between the two groups (group A 3.6 days vs group B 3.2 days). CONCLUSION: Omitting routine placement of throat packs in cleft lip/palate surgery was not associated with an increased early postoperative complication rate. Therefore, the traditional, routine placement of a throat pack during cleft lip/palate surgery can be questioned. CLINICAL RELEVANCE: The traditional, routine placement of a throat pack during cleft lip/palate surgery can be questioned.

The Cleft Aesthetic Rating Scale for 18-Year-Old Unilateral Cleft Lip and Palate Patients: A Tool for Nasolabial Aesthetics Assessment.

OBJECTIVE: To develop a reliable and easy-to-use method to assess the nasolabial appearance of 18-year-old patients with unilateral cleft lip and palate (CLP). DESIGN: Retrospective analysis of nasolabial aesthetics using a 5-point ordinal scale and newly developed photographic reference scale: the Cleft Aesthetic Rating Scale (CARS). Three cleft surgeons and 20 medical students scored the nasolabial appearance on standardized frontal photographs. SETTING: VU University Medical Center, Amsterdam. PATIENTS: Inclusion criteria: 18-year-old patients, unilateral cleft lip and palate, available photograph of the frontal view. EXCLUSION CRITERIA: history of facial trauma, congenital syndromes affecting facial appearance. Eighty photographs were available for scoring. MAIN OUTCOME MEASURES: The interobserver and intraobserver reliability of the CARS for 18-year-old patients when used by cleft surgeons and medical students. RESULTS: The interobserver reliability for the nose and lip together was 0.64 for the cleft surgeons and 0.61 for the medical students. There was an intraobserver reliability of 0.75 and 0.78 from the surgeons and students, respectively, on the nose and lip together. No significant difference was found between the cleft surgeons and medical students in the way they scored the nose ( P = 0.22) and lip ( P = 0.72). CONCLUSIONS: The Cleft Aesthetic Rating Scale for 18-year-old patients has a substantial overall estimated reliability when the average score is taken from three or more cleft surgeons or medical students assessing the nasolabial aesthetics of CLP patients.

Cost analysis of microtia treatment in the Netherlands.

INTRODUCTION: Ear reconstruction for microtia is a challenging procedure. Although analyzing esthetic outcome is crucial, there is a paucity of information with regard to financial aspects of microtia reconstruction. This study was conducted to analyze the costs associated with ear reconstruction with costal cartilage in patients with microtia. METHODS: Ten consecutive children with autologous ear reconstruction of a unilateral microtia were included in this analysis. All patients had completed their treatment protocol for ear reconstruction. Direct costs (admission to hospital, diagnostics, and surgery) and indirect cost (travel expenses and absence from work) were obtained retrospectively. RESULTS: The overall mean cumulative cost per patient was €14,753. Direct and indirect costs were €13,907 and €846, respectively. Hospital admission and surgery cover 55% and 32% of all the costs, respectively. DISCUSSION: This study analyzes the costs for autologous ear reconstruction. Hospital admission and surgery are the most important factors of the total costs. Total costs could be decreased by possibly decreasing admission days and surgical time. These data can be used for choosing and developing future treatment strategies.

Internationally adopted children with cleft lip and/or palate: A retrospective cohort study.

OBJECTIVE: The treatment approach for internationally adopted children with cleft lip and/or palate differs from locally born children with cleft lip and/or palate. They are older at initial presentation, may have had treatment abroad of different quality, and are establishing new and still fragile relationships with their adoptive parents. The aim of this study was to describe the characteristics and initial care and treatment of this group. METHODS: A retrospective cohort study was performed including all internationally adopted children with cleft lip and/or palate presenting to the cleft team outpatient clinic in the Wilhelmina Children's Hospital between January 1994 and December 2014. Medical records of all patients were reviewed; information concerning demographic characteristics, characteristics at initial presentation, and treatment were obtained. RESULTS: A total number of 132 adopted patients were included: 15% had cleft lip, 7% had cleft palate, and 78% had cleft lip and palate. The average age at the time of adoption was 26.5 months. In most cases, China was the country of origin. Seventy-eight percent had surgery in their country of origin, primarily lip repair. Fistulae in need of revision surgery were found in 8% of the patients. Pharyngoplasty was needed in 48% of the patients. No significant differences were found for mean age at adoption, gender, cleft type, and one- or two-stage palatal closure. CONCLUSION: Internationally adopted children with cleft lip and/or palate are a very diverse group of patients with challenging treatment. These children undergo surgery late and frequently need additional surgery.

Hearing sensitivity in adults with a unilateral cleft lip and palate after two-stage palatoplasty.

OBJECTIVE: To evaluate long-term hearing and middle ear status in patients treated for a unilateral complete cleft lip and palate (UCLP) by two-stage palatoplasty. METHODS: Forty-nine UCLP patients aged 17 years and older were included in this retrospective study. Patients were invited for a multidisciplinary long-term follow-up of their treatment at a tertiary center for craniofacial surgery in the Netherlands. ENT assessment included tympanometry and pure-tone audiometry. Medical files were searched for medical and surgical history. RESULTS: In total, 19.4% of the patients had significant long-term hearing loss (PTA > 20 dB), comprising conductive hearing loss in 21.5% of the patients. In the majority (70%), this hearing loss was more pronounced at higher frequencies. In 25% the high fletcher index showed hearing thresholds above 20 dB. Ventilation tubes were placed at least once in 78.7% of the patients. The frequency of tube insertion was positively correlated with the incidence of reduced tympanic compliance (tympanogram type B) and the need for a pharyngoplasty. CONCLUSION: The present study reports long-term hearing outcomes in UCLP patients with hard palate closure at 3 years of age. Persistent hearing loss was observed in 19.4% of our patients (PTA > 20 dB), mainly at the higher frequencies. Ventilation tube placement was associated with reduced tympanic compliance and higher risk on hearing loss (PTA air conduction). Our high incidence of velopharyngeal incompetence during early childhood, which is likely associated with Eustachian tube dysfunction, might have contributed to these results.