COVID-19-Associated Rhinocerebral Mucormycosis, an Incidental Finding or a Matter of Concern - Mixed-Method Systematic Review.

With the advent of COVID-19, the number of patients diagnosed with mucormycosis has increased, especially in developing countries. The reason behind this increase is that COVID-19 causes hypoxia that promotes the growth of fungus. To identify the association between mucormycosis and COVID-19, in critically ill or immunocompromised COVID-19 patients. The literature included in the review was researched from October 1, 2021, to November 1, 2022, by using the Google Scholar database as the search engine. Of the 20 articles included, there were 4 case reports, 2 case series, 10 narrative reviews, and 4 quantitative studies. Mucormycetes growth is caused by several factors, including hyperglycemia owing to previously existing diabetes or excessive use of steroids, increased ferritin levels owing to the inflammatory cascade initiated by COVID-19, and immunosuppression caused by the use of steroids or other immunosuppressive therapy. Reduced white-cell count and activity in COVID-19 leads to increased germination of fungal spores hence developing a catastrophic picture of rhinocerebral mucormycosis. Considering that the hematological patient is frequently treated with cortisone, immunosuppressed due to the underlying condition, but also through the administered therapy, the association with a possible diabetes makes this patient susceptible to developing rhinocerebral mucormycosis during COVID-19 infection. Despite being severe, the association between mucormycosis and COVID-19 is specific and treatable. Development of mucormycosis in hematological patients suffering from severe COVID-19 disease is dangerous, yet not compulsory and can be prevented. Using a common steroid-dose protocol with hyperbaric oxygen and necessary preventive measure reveals the disease as a superadded infection. Hypoxia, poor glycemic control and overuse of steroids or immunosuppressive drugs cause it.

Materials for ossicular chain reconstruction: History and evolution (Review).

The middle ear represents the anatomic space between the external auditory canal and the inner ear (Cochlea). It is comprised of the tympanic membrane, the ossicular chain [malleus (hammer), incus (anvil) and stapes (stirrup)] with the corresponding muscles and ligaments and the cavity of the middle ear. The main function of the middle ear is to convey vibratory energy (sound pressure) from the air to the cochlear fluids of the internal ear via the ossicular chain. Tympanoplasty represents a number of procedures used to re-establish the continuity of sound transmission from the tympanic membrane to the inner ear. Ever since the beginning of otologic surgery, various materials have been tested for ossicular chain reconstruction (OCR). The present review aimed to present, in a chronological sequence, the evolution of knowledge regarding this field of medicine, and to also discuss the advantages and disadvantages of different materials and designs of ossicular prostheses. The constant search for more efficient, easily tolerated and lighter materials has improved the acoustic rehabilitation process and has markedly reduced the rate of functional failure of these small prostheses.

Rare Gene Mutations in Romanian Hypoacusis Patients: Case Series and a Review of the Literature.

(1) Background: In this paper, we report on three cases of hypoacusis as part of a complex phenotype and some rare gene variants. An extensive review of literature completes the newly reported clinical and genetic information. (2) Methods: The cases range from 2- to 11-year-old boys, all with a complex clinical picture and hearing impairment. In all cases, whole exome sequencing (WES) was performed, in the first case in association with mitochondrial DNA study. (3) Results: The detected variants were: two heterozygous variants in the TWNK gene, one likely pathogenic and another of uncertain clinical significance (autosomal recessive mitochondrial DNA depletion syndrome type 7-hepatocerebral type); heterozygous variants of uncertain significance PACS2 and SYT2 genes (autosomal dominant early infantile epileptic encephalopathy) and a homozygous variant of uncertain significance in SUCLG1 gene (mitochondrial DNA depletion syndrome 9). Some of these genes have never been previously reported as associated with hearing problems. (4) Conclusions: Our cases bring new insights into some rare genetic syndromes. Although the role of TWNK gene in hearing impairment is clear and accordingly reflected in published literature as well as in the present article, for the presented gene variants, a correlation to hearing problems could not yet be established and requires more scientific data. We consider that further studies are necessary for a better understanding of the role of these variants.

Some Manifestations of Tuberculosis in Otorhinolaryngology - Case Series and a Short Review of Related Data from South-Eastern Europe.

Tuberculosis is a disease of global outreach that may affect the entire human body but is most commonly located in the lungs. Otorhinolaryngological manifestations of tuberculosis are rare, mostly occurring secondary to pulmonary disease but nevertheless represent significant diagnostic challenges. Nasopharyngeal tuberculosis is rare, representing around 1% of all upper air-way localizations and the most common presentation is in the form of adenoids. Tuberculous glossitis (oral tuberculosis) is even scarcer and may present in various clinical forms, usually mimicking a malignant neoplasm, or, less often, trauma or other infectious lesions. Oropharynx tuberculosis is usually misdiagnosed as hypertrophic chronic tonsillitis. We present four rare cases of ENT tuberculosis, primary adenoiditis and tonsillitis in a 13-year-old girl, a curious case of tuberculous glossitis in a 65-year-old woman, clinically diagnosed as a lingual neoplasm and two cases of tuberculous lymphadenopathy uncommonly located in the submandibular and supraclavicular regions. A comprehensive review of literature follows the case presentations. Tuberculous manifestation in the ear, nose and throat area remains a difficult diagnosis to establish, particularly because of its rarity and non-specific clinical appearance, and should be included in the differential diagnosis of pharynx lesions. An early diagnosis is essential to avoid occurrence of complications.

Rare and unusual benign tumors of the sinonasal tract and pharynx: Case series and literature review.

There are a number of benign tumors of the nose and pharynx that are seldomly reported in literature but that can sometimes prove difficult to treat and extremely important for differential diagnosis. The present study presents cases of rare benign tumors localized in the pharynx, nasal and sinus cavities, as well as reviews of literature and historical references for each type of tumor. Unilateral nasal hemangioma in a 72-year-old male which, although not a rare pathology, raised problems due to auto-resection of the tumor. The surgeon was able to pull it out with ease without bleeding; it is possible that the mass would have eventually fallen out. Pilomatrixoma is a relatively uncommon ectodermal benign tumor of the skin derived from hair matrix cells. Surgical resection is curative but recurrence is possible (≤5% risk). The presented case is of a 26-year-old female with a pilomatrixoma of the left cheek who, for aesthetic reasons, refused a classical external surgical approach. Trans-oral resection was performed, which proved feasible but laborious and prone to recurrence. Inverted Schneiderian papilloma is a rare benign tumor of the nasal and sinus cavities with increased potential for invasion, recurrence and malignant transformation compared with other types of papilloma and other benign tumors of the area. The tumor represents 0.5-4.0% of all nasal tumors and has been described under different names, such as villiform cancer and cylindrical/transitional papilloma. The present study reports a rare case of bilateral papilloma in a 68-year-old male. He presented with bilateral evolving nasal obstruction and hyposmia. Following surgery, the patient was treated by a multidisciplinary team and followed by a respiratory rehabilitation program.

Analysis of long-term anatomic results of radical mastoidectomy.

A long-term, retrospective, non-controlled study was performed on the drainage results of mastoidectomy (both radical and modified radical) and the relevant statistical factors that could influence the anatomic outcome were defined. The present study took into consideration the same cohort of 200 patients we have communicated with before in our previous studies concerning the long-term functional results of mastoidectomy and long-term results of ossicular replacement with biovitroceramic prosthesis. The patients were clinically followed for the same period of 8.12 years. The drainage (anatomic) results, similar to previously published functional results, were defined by analytical function of the severity and the period of evolution of disease. The main goal was to define the situations and factors (presence of complications, type of disease, type of tympanic perforation or status of ossicular chain) that influenced the drainage results that could provide us with some type of anatomical prognosis. The follow-up started at the moment of complete epithelization for each cavity as time represents the main study comparison criteria. Drainage failure was assessed by the number of otorrhea episodes. It was concluded that practically and ideally, a maximum of 84% of the mastoid and petrous cells can be cleaned out. The results of 78% drainage success are congruent to this theory. The remaining 16% of cells may contain irreversible lesions.

Analysis of long-term functional results of radical mastoidectomy.

We performed a long-term, retrospective, non-controlled study on the functional results of mastoidectomy (both radical and modified radical) and defined the relevant statistical factors that may influence the hearing outcome. In a cohort of 200 patients, we performed both radical and modified radical mastoidectomy (MRM) and followed them clinically for a period of 8.12 years. The functional results were defined by the analytical function of the severity and the period of evolution of the disease. These parameters were defined by assessing the pre-operative absolute hearing threshold (AHT), bone conduction threshold (BCT) and age of the patient at the moment of the operation. The two parameters evolved inversely proportional to the functional results and represented a complete and precise analytical tool. The global average hearing gain ratio was 32% and the ratio for unmodified pre-operative hearing (status quo ante) was 61%. With favorable prognostic factors, the average gain rate was 56% and the hearing-loss rate was 5% (1-dB SPL nominal value). The maximum ratio for gain was 81% and for hearing loss this was 0%.

Prevalence of GJB2 gene mutations correlated to presence of clinical and environmental risk factors in the etiology of congenital sensorineural hearing loss of the Romanian population.

Although etiologically heterogeneous at least 50% of all early on-set hearing losses have a genetic cause and of these, the large majority, 75-80% are most probably autosomal recessive and 70% are non-syndromic. The rest of the congenital hearing losses are determined by clinical and environmental factors such as ototoxic medication, prematurity, and complications at birth. During the last decade it became clear that 50-80% of all such afflictions result from mutations in a single gene, GJB2, which encodes the protein Connexin 26. In order to, at least partially clarify this problem, especially in an emerging country such as Romania, where the problem is not studied adequately, we developed a comprehensive study of genetic, clinical and environmental risk factors for congenital hearing loss. The two most common variations of this gene, 35delG and W24X in children with positive diagnosis of bilateral severe to profound sensorineural hearing loss were investigated. A cohort of 34 children (20 female and 14 male), ages between 2 and 10 (mean age 4.62 years), coming from 33 non-related families were evaluated. All cases were diagnosed with severe or profound bilateral congenital SNHL. A statistical comparison of genetic and environmental/clinical prevalence was also attempted since the presence of a genetic disorder cannot rule out the role of other documented risk factors in the etiology of SNHL. The results showed that, 29.4% of cases (10/34) were homozygotic for the 35delG mutation 35delG/35delG), also known as genotype Δ/Δ. 5.88% of cases (2/34) belong to the heterozygotic bi-genic group 35delG/W24X. The clinical factors with high statistical significance for SNHL in a non-genetic group have no significance for genetic SNHL patients. Thus, the present study confirms the relatively high prevalence of the 35delG and W24X mutations in cases of congenital non-syndromic severe of profound bilateral SNHL.

Long-term histological results of ossicular chain reconstruction using bioceramic implants.

Middle ear problems are addressed using tympanoplasty, which requires a mobile tympanic membrane and secure sound-conducting mechanism. The present is a long-term, retrospective, non-controlled study on the results of ossiculoplasty using bioceramic implants of autochthonous origin and which defined the statistical relevant factors that could influence the rate of implant rejection. In a cohort of 108 patients ossiculoplasties with bioceramic implants were performed and patients were followed up clinically for a period of minimum 7 years. Several factors were identified that could influence the results of the implantation and the statistical correlations were studied. The rejection rate after 9.12 years was 21% (23 patients). Histological integration rate was 79% (85 patients), similar to results reported in literature for both bioceramic and titanium implants. Hydroxyapatite has many of the ideal characteristics required to be a good prosthesis with a high degree of biocompatibility, very low extrusion rate, low risk of disease transmission and good functional results. Although perhaps considered of historical interest, bioceramic implants are cheaper and can be produced locally, which is a great advantage for struggling economies.