Systematic Review of Current Audiological Treatment Options for Patients with Treacher Collins Syndrome (TCS) and Surgical and Audiological Experiences of an Otorhinolaryngologist with TCS.

Treacher Collins syndrome (TCS) is a rare congenital craniofacial condition that affects approximately one out of fifty thousand births. Different ratios of TCS patients have conductive hearing loss: 88%1 vs. 91.4-100.00%2. For this reason, it was examined which hearing solutions can be used with this condition and how effective they are. A systematic literature review was conducted, which showed that the bone-anchored hearing aid (BAHA, OSIA), the bone conduction implant (Bonebridge) or the active implant of the middle ear (Soundbridge) are reliable methods for the treatment of conductive hearing loss in TCS patients. After the implantation of all available hearing solutions, improved hearing and speech comprehension were observed. Additionally, a statement regarding the treatment of TCS and a personalized point of view of a clinical expert with TCS were provided. However, due to the small amount of data, no general recommendations can be given for the treatment of hearing loss in TCS patients; therefore, it is advised to collect more data on hearing solutions for TCS patients in future research.

The usefulness of wideband absorbance in the diagnosis of otosclerosis.

Objective: To compare wideband absorbance (WBA) patterns between ears with otosclerosis and normal hearing ears and to investigate if WBA findings could be useful in the diagnosis of otosclerosis.Design: WBA was obtained at 107 frequency samples ranging from 0.226 to 8 kHz (24 per octave). A T-test was performed to compare between WBA in ears with otosclerosis and in normal hearing ears. The ability of WBA to discriminate between the patients with otosclerosis from the normal hearing participants was tested with a receiver operating characteristics (ROC) curve analysis.Study sample: Thirty-five patients with otosclerosis (age 31-64) and thirty-five normal hearing volunteers (age 32-64).Results: In frequency range 0.432-1.059 kHz, mean WBA in otosclerosis was significantly lower than mean WBA in normal hearing ears and in frequency range 4.238-8 kHz mean WBA in otosclerosis was significantly higher than mean WBA in normal hearing ears. The ROC analysis revealed that ears with otosclerosis and normal hearing ears could be distinguished based on mean WBA in frequency range >0.5 ≤ 1 kHz (AUC = 0.673) and based on mean WBA in frequency range >4 ≤ 8 kHz (AUC = 0.769).Conclusion: Our results suggest that WBA findings in ears with otosclerosis differ from WBA findings in normal hearing ears.

ACTIVE MIDDLE EAR VIBRANT SOUNDBRIDGE SOUND IMPLANT.

The Vibrant Soundbridge represents a new approach to hearing improvement in the form of active implantable middle ear hearing device. Unlike conventional acoustic hearing aids, which increase the volume of sound that goes to the eardrum, the Vibrant Soundbridge bypasses the ear canal and eardrum by directly vibrating the small bones in the middle ear. Because of its design, no portion of the device is placed in the ear canal itself. The Vibrant Soundbridge has been approved by the FDA as a safe and effective treatment option for adults with moderate to severe sensorineural, conductive or mixed hearing losses who desire an alternative to the acoustic hearing aids, for better hearing. The paper presents a review of the active middle ear implant Vibrant Soundbridge, which has been also implanted at the Department of Otorhinolaryngology and Head and Neck Surgery, Sestre milosrdnice University Hospital Center, which is the Referral Center for Cochlear Implantation and Surgery of Hearing Impairment and Deafness of the Ministry of Health, Republic of Croatia.

A Rare Case of Recurrent Mucoepidermoid Carcinoma of the Nasal Vestibule.

We report a rare case of a large recurrent mucoepidermoid carcinoma (RMEC) in an 81-year-old female smoker, which has originated in the right nasal vestibule. The recurrent tumour was inadequately treated for 6 years. It was a slow-growing tumour for 3 years and then began to enlarge at a higher pace. In the next three years it has covered a large part of the face. The patient had refused any medical treatment. The tumour caused breathing and swallowing difficulties. Because of the profuse bleeding from the tumour, the patient underwent emergency surgery. Surgical treatment consisted of rhinectomy and resection of the central upper lip and part of the right cheek. The facial defect was reconstructed immediately. Recovery from surgery was fast with no complications. Postoperative Multislice Computed Tomography scan showed no metastases so the patient did not receive any chemotherapy or radiotherapy. During a 2.5 years' follow-up period there was no recurrence of the disease.

Hyperbaric Oxygen Therapy as Salvage Therapy for Sudden Sensorineural Hearing Loss.

OBJECTIVE: To measure the effect of hyperbaric oxygen (HBO) therapy as salvage therapy after the failure of steroid therapy for sudden sensorineural hearing loss (SSNHL). MATERIALS AND METHODS: Ninety-three patients with SSNHL were unsuccessfully treated with systemic steroid therapy. Following steroid therapy, 43 patients received additional HBO therapy while 50 did not. Hearing levels at 0.25, 0.5, 1, 2, 4, and 8 kHz before and after therapy were measured. RESULTS: A significant difference in hearing thresholds after HBO therapy was found at all frequencies in patients with a hearing loss of >61 dB. The group of patients with a hearing threshold of ≤60 dB had a significant improvement only at 250 and 500 Hz, while group of patients without additional therapy control group showed no hearing improvement. CONCLUSION: Hyperbaric oxygen therapy therapy as salvage therapy for SSNHL showed some benefits in hearing improvement. Better results could be expected in patients with severe hearing loss, while in patients with mild-or-moderate hearing loss, recovery should be expected only at low frequencies.

Significance of intraoperative findings in revision tympanomastoidectomy.

PURPOSE: The study was designed to assess correlations between intraoperative findings in revision tympanomastoidectomy as predictors of cholesteatoma recurrence. MATERIALS AND METHODS: A retrospective single-institution cohort of 101 patients who underwent surgical treatment for recurrent chronic otitis media in a tertiary referral otology centre. RESULTS: Out of 101 patients, 65 had canal wall up and 36 canal wall down revision surgery. There were 35 cholesteatoma recurrences. Sites most commonly associated with recurrent disease were residual facial ridge cells in 46 (45.5%), ossicular chain sites in 46 (45.5%) patients, posterior external auditory canal wall erosions in 38 (37.6%) patients and mastoid apex recurrence in 35 (34.7%) patients. Ossicular and posterior external auditory canal wall erosion and incomplete removal of mastoid apex cells correlate well with cholesteatoma recurrence accompanied by canal wall up surgery (p=0.009). Residual mastoid apex cells, posterior external auditory canal wall erosion and presence of residual facial ridge cells were identified as the strongest positive predictors of cholesteatoma recurrence, identifying high risk patients associated with canal wall down procedures (p=0.0036). CONCLUSIONS: Correlations between intraoperative findings and cholesteatoma recurrence could improve preoperative and intraoperative planning and reduce the rates of postoperative failures1 due to mismanagement of high risk areas.

Primary Ameloblastoma of the Temporal Bone.

Ameloblastoma is a locally aggressive tumor derived from odontogenic epithelium. Although benign, its clinical behavior can often exhibit malignant characteristics. It is marked by slow and persistent growth with infiltration of adjacent tissues. Almost 70% occur in the mandible in patients older than 30 years. Recurrence of ameloblastoma from inadequate treatment is frequent. Because of its slow growth, recurrences can present decades after primary surgery. A primary ameloblastoma in an area outside the mandibular, maxillary, and infratemporal fossa regions has not been described in detail to date, with only 1 possible case mentioned in the literature. The authors present a case of primary temporal bone ameloblastoma in a 17-year-old boy. The tumor originated in the left mastoid, infiltrated the lateral semicircular canal, facial nerve, and cochlea, and adhered to the sigmoid sinus and posterior cranial fossa dura. Although invasion of multiple structures in the infratemporal fossa and temporal bone leads to variable disease presentation, this case is unique because the first symptom of disease was sudden and recurring unilateral sensorineural hearing loss. Surgery required transection of the facial nerve. Histopathology confirmed primary temporal bone ameloblastoma. The difficulties in achieving wide surgical margins, diagnostics, and further management are addressed.

Therapeutic efficacy of azithromycin and acetylcysteine in chronic otitis media with effusion.

Otitis media with effusion (OME) is a common disease in childhood. There is no consensus on the optimal therapeutic option for OME. Considering the known efficacy of acetylcysteine (AC) and azithromycin (AZ) in the treatment of middle ear mucosa, the aim of the study was to assess their efficacy in the management of chronic OME. The study included 90 children with OME, both ears. They are divided into three groups of 30 children. Group 1 (AC) patients were treated with acetylcysteine per os, 3 × 100 mg, for 3 weeks; group 2 (AZ) with body weight adjusted dose of azithromycin for 3 days; and group 3 (AC + AZ) with a combination of acetylcysteine and azithromycin at doses described above. Three measurements were performed. On second measurement, tympanogram improvement was recorded in 45% of 60 ears in group I, 53.3% of 60 ears in group II, and 61.7% of 60 ears in group III. The percentage of improvement was highest in group III. Although between-group differences did not reach statistical significance, the results obtained appear to be clinically relevant. In conclusion, conservative therapy for chronic OME is reasonable. Although study results don't have a strong statistical differences and may not refer clinical improvement results suggest that this combination of drugs (antibiotics, bronchosecretolytics) can be useful in the treatment of OME.

Clinical and audiological findings in children with acute otitis media.

CONCLUSION: It is recommended to perform follow-up tympanometry and if necessary tonal audiometry in children who have normal plain otoscopy findings after recovering from acute otitis media (AOM). Children with Type B tympanogram, 3 months following the onset of AOM, are very likely to have a conductive hearing loss. Type B tympanogram is a much better indicator of effusion in the middle ear compared to plain otoscopy. OBJECTIVE: This study was undertaken to investigate the frequency and duration of middle ear effusion in children following an episode of acute otitis media, to track changes in tonal audiometry and tympanometry findings in the post-AOM period, and recognize the optimal timing for performing both tests. METHODS: In this study, 125 children aged 5-7 years with bilateral AOM were randomly selected and separately followed up for 3 months. The children underwent six ear, nose, and throat (ENT 1-6) examinations, six tympanometries (TM 1-6), and three tonal audiometries (TA 1-3). Evaluation of nasopharynx was done at the ENT 1 examination. Children who received ventilation tubes were followed for 21 month altogether. RESULTS: At the first otoscopy, pathological findings were recorded in 250 ears/125 children (100.0%). The number of pathological otoscopy findings decreased at each subsequent examination. At ENT 6 all children had normal otoscopy findings. Type B tympanogram was detected in 49/250 (19.6%) ears at TM 6, performed 3 months following the onset of the disease. At the TA 1 conductive hearing impairment was recorded in 158/250 (63.2%) ears, at TA 2 in 66/250 (26.4%), and at TA 3 in 39/250 (15.6%). Most of them were associated with Type B tympanogram.

[CROATIAN GUIDELINES FOR DIAGNOSIS AND MANAGEMENT OF BENIGN PAROXYSMAL POSITIONAL VERTIGO (BPPV)]. / HRVATSKE SMJERNICE ZA DIJAGNOSTIKU I LIJECENJE BENIGNOGA PAROKSIZMALNOG POZICIJSKOG VERTIGA (BPPV-A).

BPPV is generally the most common cause of vertigo, caused by a pinch-off of tiny calcium carbonate crystals (called the otoconia or the otoliths) from the macula utriculi, most frequently due to the degenerative processes or a trauma, whereby the crystals, under the action of gravity in certain head positions coinciding with its direction, arrive to some of the semicircular canals, usually the posterior one, due to the existent anatomical circumstances and relationships, thus creating an inadequate stimulus of the cupular senses while floating through the endolymph and provoking symptoms of a strong and short-term dizziness. Two main clinical forms can be distinguished: canalolythiasis, with an accommodation of otolithic debris in the semicircular canal, and cupulolythiasis, with their location immediately next to the cupular sense. The diagnosis is established by a positive positioning test, Dix-Hallpike for the posterior and the supine roll for the lateral canal. Although one can expect a spontaneous recovery subsequent to few weeks or months, various methods of otolith repositioning to a less sensitive place lead to a prompt improvement while reducing or withdrawing the symptoms completely. These guidelines are intended for all who treat the BPPV in their work, with an intention to assist in the diagnosis and application of an appropriate therapeutic method.

Radiotherapy-induced hearing loss in patients with laryngeal and hypopharyngeal carcinomas.

The purpose of this study was to investigate a hypothesized correlation of development of a sensorineural hearing loss and radiotherapy in patients with laryngeal and hypopharyngeal carcinoma. This prospective study included a total of 50 patients, which after strict exclusion critera (audiologic problems before RT primary tumors of the auditory system, spread of the primary tumor to any part of the auditory system) resulted in 23 analyzed patients, ranging between 50 and 76 years of age, with a mean age of 60. Audiometry measuring frequency-specific thresholds was performed in three time points: one month before radiotherapy, one and six months after radiotherapy. A significant statistical difference in hearing tresholds after radiotherapy was found in 6 out of 23 patients. An obvious tendency towards hearing loss without statistical significance at 250 and 4000 Hz was found for a whole tested population (p < or = 0.3 with Bonferroni correction). Observed tendency towards hearing loss after radiotherapy of laryngeal carcinoma was related to side of the tumor and less severe when chemotherapy was not added as adjuvant therapy. These results should help to decrease a rate of hearing loss by careful planing of ear protection, by using observed frequencies as relevant markers of hearing loss and by reconsidering adjuvant chemoterapy during radiotherapy of laryngeal carcinoma.

Nasopharyngeal bacterial flora in healthy preschool children during winter-spring months.

The paper aimed to determine the incidence of colonization of Streptococcus pyogenes, Streptococcus pneumoniae, Haemophilus influenzae type b and Neisseria meningitidis in the nasopharynges of healthy children in two preschool institutions during winter and spring months, without using antimicrobial treatment or serotyping of these bacteria. In addition to colonization of the above bacteria, the research that continued for 3 months monitored the length of their persistence in and disappearance from children's nasopharynges, children's health statuses, and provision of adequate medical interventions in children demonstrating clinical signs of disease. The ultimate aim of the paper was based on contributing to clearer and more accurate determination of a medical procedure in case of a positive result for bacteria intended to be found in the nasopharynx of a healthy child who spends time in a preschool institution.

The developmental characteristics of mastoid pneumatisation in cleft palate children: the genetic influence.

Physiologic and developmental role of mastoid pneumatisation in children with otitis media with effusion (OME) is still controversial. For measuring mastoid pneumatisation and examine developmental characteristics, we used children with orofacial malformation of high risk for long term negative pressure in the middle ear and are expected to have lower rate of size and growth of pneumatisation. Mastoid were measured on Schuller's mastoid X-ray pictures planimetrically in study group of 146 children with bilateral (BCLP), unilateral (UCLP) and isolated (ICP) cleft palate, and control group of non-cleft 52 children, both groups with confirmed otitis media with effusion and no previous otological surgery. The lowest pneumatisation found in BCLE, BCLP and UCLP showed no growth of mastoid with age and lower mastoid size than OME controls. ICP is the only cleft type with growth of mastoid with aging. OME patients has the highest size of mastoid and growth rate with aging.

Benign paroxysmal vertigo in childhood.

The aim of the paper is to describe the clinical picture of benign paroxysmal vertigo (BPV) in childhood. BPV in childhood often goes unrecognized in spite of the fact that vertigo and balance disorders are not uncommon in children. Four cases are described with clinical examination findings between the attacks.

Long-term functional outcomes after 10 years of bilateral cochlear implantat use.

The aims were to determine the benefit of bilateral cochlear implantation in a 20 years old patient implanted in Croatia on hearing and speech development. The male patient, after 10 years of deafness, got cochlear implants Med-EL Combi 40+ on both sides in one-stage surgery. The etiology of his deafness was posttraumatic meningitis. Auditory capacity and speech recognition tests were performed for both ears separately and together Average hearing level on the right ear with right cochlear implant switched on started at 62 dB 1 month after the cochlear implantation and was on 55 dB after 10 years. Average hearing level on the left ear with left cochlear implant switched on started at 55 dB 1 month after the cochlear implantation and was on 32 dB after 10 years. Average hearing level on the both ears with 2 cochlear implants switched on started at 35 dB 1 month after the cochlear implantation and was on 27 dB after 10 years. Long-term functional outcomes with bilateral cochlear implantation provides advantages over unilateral implantation including improved hearing level, speech perception in noise and improved sound localization.

Differences between electrically evoked compound action potential (ECAP) and behavioral measures in children with cochlear implants operated in the school age vs. operated in the first years of life.

OBJECTIVE: The aim of this study was to identify the differences in the NRT measures, behavioral measures, and their relationship between the group of congenitally deaf children operated in the first years of life and the group of children operated in the school age. METHODS: The study included 40 congenitally deaf children with cochlear implants divided into two groups. Group 1 was composed of 20 children (mean age at operation 2.3 years, range 1.4-4.6 years) and Group 2 was composed of 20 children (mean age at operation 11.3 years, range 7.0-17.1 years). The ECAP was recorded using the Nucleus 24 neural response telemetry (NRT) system. In each child, the responses were evoked by the apical, middle and basal electrodes. The analyzed parameters were: the ECAP threshold (T-NRT), N1P2 amplitude, N1 latency, slope of the amplitude growth function, response morphology, threshold (T-) level, maximum comfort (C-) level, dynamic range (DR), T-NRT as a percentage of the map DR, the correlation between the T-NRT and the T- and C-levels. The recordings of parameters were performed two years after implantations. RESULTS: The T-NRT, DR, T-NRT as a percentage of the map DR and the correlation between T-NRT and C-levels were significantly different between both groups of children. There were no statistically significant differences between the groups with respect to the amplitude, latency, slope and morphology recorded using the same electrodes. However, intragroup differences regarding NRT measures and behavioral measures with respect to the position of stimulating electrode were more prominent in Group 2 than in the Group 1. CONCLUSIONS: Results of this study have also found a great variability of NRT and MAP measures within and across patients in both groups of children, but it was still more pronounced in the group of school children. NRT profile across electrodes follows MAP profiles better in the Group 1 then in the Group 2. Overall findings of NRT and MAP measures are not consistent and unambiguous as we expected, but still suggest potential differences between results in children operated in first years of life, and those operated in school age.

Hidradenoma of the external auditory canal: clinical presentation and surgical treatment.

The aim of this article is to present clinical features, diagnostic procedures and surgical treatment of a rare ear tumor. We report a case of 78 year old female with hidradenoma of the external auditory canal. Patient had a sensation of pain and fullness with permanent ottorhea from the right ear for one year Temporal bone computed tomography showed a tumor of the external ear, 6 centimeters in diameter, without bone, temporomandibular joint or intracranial invasion; the tumor was limited medially by the tympanic membrane. Biopsy was performed and pathohistology finding was: hydradenoma nodulare atypicum. Surgical intervention and wide tumor removal in general endotracheal anesthesia was performed. One year after the surgery there was no sign of tumor recurrence. Hidradenoma is rare ear tumor arising from the epithelial cells of sweat glands of the external auditory canal. Radiological evaluation and pathohistology confirmation of hidradenoma is necessary and wide excision of the tumor is the treatment of choice.