Management of children with auditory neuropathy spectrum disorder (ANSD) / Tratamento de crianças com espectro da neuropatia auditiva (ENA)

ABSTRACT INTRODUCTION: ANSD is a challenging problem. OBJECTIVE: To present our experience on management of the children with ANSD with respect to clinical data. METHODS: This retrospective study included all children younger than 16 years of age who applied to the department between 2005 and 2013 (with the exception of newborn hearing screening NHS referrals). The data were derived from pure tone, OAEs and ABR tests, and further medical risk factors of the subjects were evaluated. RESULTS: ANSD was recognized in 74 ears of 40 children (B/U: 34/6) among 1952 children with SNHL (2.04%) detected among 9520 applicants to the department (0.42%). The clinical tests revealed that hearing loss greater than 15 dB was present in both ears of 38 cases. The degree of hearing loss was profound in 48% children, severe in 12% children, moderate in 28% children, mild in 10% children and normal in 5% children. ABRs were absent/abnormal in 37/3 ears and CMs were detected in all. Acoustic reflexes were absent in all ears. Rehabilitation was managed by CI and hearing aids in 15 and 23 cases, respectively. FM system was given to two cases displaying normal hearing but poor speech discrimination in noisy environments. CONCLUSION: ANSD is a relatively challenging problem for the audiology departments because of its various clinical features and difficulties in management. Our patients with ANSD most commonly displayed profound hearing loss. The number of overlooked cases may be minimized by performing ABR and OAE in every case referred with the suspicion of hearing loss.

Resumo Introdução: Espectro da neuropatia auditiva ainda é uma condição clínica desafiadora. Objetivo: Apresentar nossa experiência no tratamento de crianças com espectro da neuropatia auditiva em relação aos dados clínicos. Método: Este estudo retrospectivo incluiu crianças menores de 16 anos de idade que deram entrada no departamento entre 2005 e 2013 (com exceção de encaminhamentos para triagem auditiva neonatal). Foram avaliados os dados obtidos a partir dos exames de audiometria tonal, emissões otoacústicas (EOA), potencial evocado auditivo de tronco encefálico (ABR) e outros fatores de risco. Resultados: Das 1.952 crianças com perda auditiva neurossensorial (2,04%) detectadas dentre os 9.520 candidatos que deram entrada no departamento (0,42%), espectro da neuropatia auditiva foi reconhecida em 74 orelhas de 40 crianças (B/U: 34/6). Os testes clínicos revelaram que uma perda auditiva superior a 15 dB estava presente em ambas as orelhas em 38 casos. O grau de perda auditiva das crianças era profundo em 48%, grave em 12%, moderado em 28%, leve em 10%, e normal em 5%. ABR estava ausente/anormal em 37/3 orelhas e microfonia coclear foi detectado em todas as crianças. Reflexos acústicos estavam ausentes em todas as orelhas. A reabilitação foi tratada com implante coclear e aparelhos auditivos em 15 e 23 casos, respectivamente. Um sistema FM foi utilizado em dois casos que apresentavam audição normal, mas discriminação deficiente da fala em ambientes ruidosos. Conclusão: Espectro da neuropatia auditiva é um problema desafiador para os departamentos de audiologia, devido às suas várias características clínicas e dificuldades no tratamento. Em nossos pacientes a perda auditiva profunda foi a mais frequente. O número de casos negligenciados pode ser diminuído com a realização dos exames ABR e EOA em todos os casos encaminhados com suspeita de perda auditiva.

Bacterial biofilm formation in the middle-ear mucosa of chronic otitis media patients.

Biofilm formation is present in the middle-ear mucosa of chronic otitis media (COM) patients and COM is a biofilm-related disease. Biofilms are organized and complex communities in which bacteria communicate to each other and gain tremendous advantages. In this unique structure, bacteria can diffuse nutrients, gain resistance to antimicrobials agents and host defense mechanisms. Recently bacterial biofilms have been proven to be important in infectious diseases of head and neck region. A prospective case-control study was conducted. The study group comprised of patients with chronic otitis media and patients undergoing surgery for cochlear implantation was involved in the control group. Study group also divided to subgroups SSA and SSB according to history of ear discharge within last six months. Direct microscopy (DM) and transmission electron microscopy (TEM) were used to assess presence of biofilms. Totally 19 patients, 10 with ear discharge history within last 6 months and 9 without discharge comprised the study group. Control group comprised of 9 patients undergone cochlear implantation. In all of the patients with ear discharge history and in two of the patients without ear discharge history, biofilm formation was detected by both DM and TEM. All control group members were free of biofilm formation. The differences were statistically significant between study and control groups (p = 0.002) and between study subgroups (p < 0.001); but not significant between study subgroup without ear discharge history and control group (p = 0.470). In the middle ear mucosa of patients with chronic otitis media, biofilm formation is common, especially when ear discharge history is present.

Surgical exposure in retrosigmoid approach: do we need cerebellar retractors?

BACKGROUND: The aim of this study was to present our technique of posterior fossa surgery and to evaluate whether cerebellar retractors are needed. METHODS: Since March 1989, more than 500 surgeries were performed through retrosigmoid approach, and our surgical technique was described. The data were evaluated whether or not cerebellar retractors were used in the operations. RESULTS: The surgeries were vestibular neurectomy, acoustic neuroma removal, and neurovascular decompression in 351, 135, and 14 patients, respectively. Cerebellar retractors have not been used in vestibular neurectomies and in most acoustic neuroma surgeries. The cerebellar retractors have been used in all of the neurovascular decompression surgeries where better exposure of the root entry zones of the cranial nerves is needed, and intracranial endoscopy is applied for further exposure and decompression of the vascular loop. CONCLUSION: There is no need for cerebellar retractors when adequate conditions such as patient positioning, adequate anesthesia techniques, and surgical manipulations are applied in posterior fossa approach except for neurovascular decompression and some of the acoustic neuroma surgeries.

Ultrastructural analysis of the chorda tympani nerve in chronic suppurative otitis media.

OBJECTIVE: To compare ultrastructure of the chorda tympani nerve by light and electron microscopy in patients with otosclerosis and chronic suppurative otitis media. STUDY DESIGN: Comparative prospective study. SETTING: Tertiary care, referral medical center. PATIENTS: The tympanic segments of chorda tympani nerves were collected for ultrastructural investigation in 20 cases with chronic suppurative otitis media and 10 cases with otosclerosis that underwent middle ear surgery. RESULTS: Histopathologic examinations of the suppurative group showed that unmyelinated fibers were almost totally lost and replaced by collagen fibers. The thickness of the myelin sheaths was very slender, whereas some of them were atrophic. A substantial increase was encountered in the endoneural collagen substance and connective tissue, whereas inflammatory elements and edema were present occasionally. Degenerative alterations of the myelinated fibers mainly occurred in the form of adaxonal vacuoles. There was disorganization and separation of parallel lamellae of Schmidt-Lanterman clefts. CONCLUSION: The chorda tympani nerve should be preserved in otologic surgery. However, inadvertent dissection of the chorda tympani nerve in chronic suppurative otitis media surgery will not possibly cause a postoperative disturbance in light of ultrastructural changes that occur in the nerve.

[The adverse effect of gas formation on prognosis in a patient with deep neck infection]. / Derin boyun enfeksiyonlu bir olguda gaz olusumunun prognoza olumsuz etkisi.

A seventy-one-year-old woman was hospitalized with a suspicion of deep neck infection and poor general health. She had been receiving treatment for hepatitis, diabetes mellitus, and cardiac failure and had a history of tooth ache and severe neck pain lasting for the past 10 days. She had been admitted to another center where she had received antibiotic treatment for five days for widespread swelling in the neck and lower extremities, fatigue, and difficulty in breathing and swallowing. Upon admission, computed tomography showed gas formation in the neck and facial regions. Prompt abscess drainage was performed and intense treatment with antibiotics was continued. Despite all efforts, the patient died on the second day of hospitalization from cardiopulmonary arrest. This case emphasizes how urgent drainage is when gas formation is detected in deep neck infections, with inevitable poor prognosis with antibiotic treatment alone.

Asymptomatic IgE mediated food hypersensitivity in patients with nasal polyps.

The aim of this prospective study is to compare the prevalence of atopy in patients afflicted by nasal polyps with the atopy prevalence in healthy volunteers without nasal polyps, since systemic allergy and allergy in the nasal mucosa are still being debated as underlying causes for nasal polyps. Thirty-four cases with nasal polyposis without asthma and history of allergy or atopic disease were enrolled in the study and compared with 20 healthy volunteer controls in respect to asymptomatic food hypersensitivity. Hypersensitivity for 48 kinds of commonly consumed food in Turkey was investigated by an epicutaneuos prick test, Multi-Test II (Lincoln Diagnostic, Inc, USA), using a special applicator. The food allergy test was positive in 25 out of the 34 cases with nasal polyps and in 6 out of the 20 controls. The difference between the two groups was statistically significant (chi2 = 0.000, p < 0.001). The number of skin tests with positive results in patients with nasal polyps ranged from 1 to 37 (mean +/- S.D. = 10.0 +/- 7.9), whereas in the control subjects the range was 1 to 10 (mean +/- S.D. = 4.0 +/- 3.3). The difference in the number of food reactions was also statistically significant. Asymptomatic food hypersensitivity, being immunologically mediated, may be a triggering factor for the pathogenesis of nasal polyps. Therefore, treatment of asymptomatic food allergy in patients with nasal polyps may alleviate symptoms, slow the progress of nasal polyps and prolong the disease-free interval after polypectomy.

Otitis media with effusion and histopathologic properties of adenoid tissue.

OBJECTIVE: Adenoidectomy is being generally used for the treatment of otitis media with effusion (OME). The purpose of current study was to determine objectively the significance of the histopathology of adenoid tissue on the development of otitis media with effusion. METHODS: The records of all the patients operated on with the diagnosis of adenoid enlargement with or without OME were reviewed and pathologic specimen were re-evaluated regarding histopathological properties by one pathologist unfamiliar with the diagnosis. Sixty-one patients with adenoid hyperplasia were enrolled in the study group, 38 males and 23 females. Age ranged between 3 and 13 years (mean age was 7.03+/-3.26 years). All the patients of study group were those operated on due to the adenoid hyperplasia and uni- or bilateral OME. Control group was composed of 39 male and 26 female patients (age range was between 3 and 13 years, and mean age was 7.06+/-3.04 years) with solely adenoid hyperplasia. RESULTS: The squamous metaplasia was present in 47 (77%) and 14 (22%) patients of study and control groups, respectively. The fibrosis of connective tissue interspersed follicles of adenoid was present in 29 (48%) and 6 (9%) patients of study and control groups, respectively. The statistical analysis revealed a significant prevalence of squamous metaplasia (P<0.001) and fibrosis of connective tissue interspersed follicles of adenoid (P<0.001) for a surgical indication of adenoid hyperplasia with OME than for without OME. The prevalence of other parameters was not significantly different between two groups. CONCLUSIONS: Adenoid tissue not only exerts an obstructive influence on the eustachian tube lumen when enlarged, but also impedes (hinders) mucociliary drainage of the middle ear by the way of non-ciliated metaplastic epithelium and fibrosis of connective tissue.

Rapidly growing sublingual dermoid cyst throughout pregnancy.

Dermoid cysts (DCs) are subcutaneously located cystic masses that contain epithelium and adnexal structures. They are most commonly located in the ovaries and sacral region. Seven percent of DCs are found in the head and neck. Sublingual DCs may develop above the mylohyoid, presenting in the floor of the mouth or below it, causing a submental or submaxillary mass. We presented a case with a sublingual giant DC enlarged rapidly during pregnancy manifesting deglutition and mild respiratory problems. The growth of a DC of the neck may be accelerated in pregnancy period and may ensue severe symptoms challenging both mother and fetus. We proposed the removal of sublingual DCs before attempting to conceive to eliminate the risk of rapid growth of DC that results in respiratory and deglutition problems.

Removal of a laryngeal leech: A safe and effective method.

This article presents a case of hoarseness, dysphagia, and hemoptysis caused by the laryngeal localization of a leech. This pathological condition is extremely rare in urban areas but is frequent in endemic rural areas. Laryngoscopic evaluation of our patient has shown a leech lodged on the left arytenoid region hanging down to larynx. We present a patient living in an urban area with laryngeal leech that was removed under general induction anesthesia of sevoflurane via a face mask without endotracheal intubation.

A case of giant cholesteatoma extending to the posterior fossa.

We present a 43-year-old male patient who developed a giant cholesteatoma with lateral sinus obliteration and involvement of the posterior cranial fossa. The only complaints were left-sided aural discharges and total sensorineural hearing loss. Examination revealed a mass lesion consistent with cholesteatoma within the left middle ear. Computed tomography and magnetic resonance imaging showed an extensive acquired cholesteatoma in the left middle ear, invading the posterior cranial fossa and leading to lateral sinus obliteration. The patient underwent left radical mastoidectomy. He had an uneventful postoperative follow-up for 13 months. This case emphasizes the value of preoperative radiologic assessment of giant cholesteatomas that may present with relatively few or limited symptoms.

The correlation between septal deviation and concha bullosa.

OBJECTIVES: To investigate the incidence of concha bullosa (CB) in patients with septal deviation and the correlation between the angle of deviation and degree of pneumatization. Study and design This study was designed as a prospective trial. METHODS: Computerized tomography scans of the paranasal sinuses of 100 consecutive patients with the chief complaint of nasal obstruction and the diagnosis of septal deviation were taken. CB, sinus diseases, and septal deviation angles were recorded. RESULTS: Angles of deviation ranged between 3 and 25 degrees (mean, 13.0 +/- 4.1 degrees). The mean of angle of deviation in cases with CB and in cases without CB was 13.0 +/- 4.0 and 13.0 +/- 4.2 degrees, respectively. No statistical difference was found between them (P > 0.05). Pneumatization of the contralateral CB was statistically significantly higher than that of ipsilateral CB (P < 0.05). CONCLUSION: Septal deviation does not appear to give rise to the formation of CB, but augments the pneumatization of the middle turbinate depending on the degree of deviation angle.