Delayed-Onset NOG Gene-Related Syndromic Conductive Deafness: A Case Report

We report a 6-year-old girl with progressive bilateral conductive hearing loss for 2 years. She passed the newborn hearing screening conducted with otoacoustic emissions testing and had a normal development of speech and language, which indicated that her deafness was delayed-onset. She also had congenital proximal interphalangeal joints. Proximal symphalangism was confirmed by genetic testing ( NOG gene: c.406C > T, p.R136C). Bilateral stapes ankyloses were proved by surgery and her hearing was improved after stapedotomy by over 30 dB. Besides, this case should remind clinicians to carefully distinguish NOG gene-related deafness from congenital ossicular malformation and pediatric otosclerosis.

Brainstem Auditory Evoked Response (BAER) in Canine Otitis Externa

The objective of this study was to assess the brainstem auditory evoked response (BAER) wave pattern in normal dogs as well as those with otitis externa (OE). The BAER test was performed on twenty five dogs with varying degrees of OE. Our study found that BAER of ears with severe OE had prolonged latencies of all waves and lower wave I to wave V amplitude ratio compared to the mildly affected or normal ears. However the inter – peak latencies were unaffected. Thus BAER can be a useful diagnostic tool to assess the hearing impairment due to conductive deafness in OE.

TO EVALUATE PRE- AND POST-OPERATIVE HEARING OUTCOME AFTER TYMPANOPLASTY: A COMPARATIVE STUDY.

Aims: The aim of this study is to evaluate hearing outcome in the pre- and post-operative after tympanoplasty Materials and Methods: The present observations study was conducted on 30 randomly selected patients visited the department of ENT, Nalanda Medical College and Hospital, Patna, Bihar, India. Thirty cases of diagnosed chronic otitis media, aged above 18 years, with a demonstrable conductive deafness not more than 40 dB was included in the study. Results: The mean age of the patients is 32.16 ± 3.61 years. Female outnumbered male counterpart. Commonest presenting complaints was Otorrhoea (93.3%) followed by Hearing loss (86.7%), Vertigo (53.3%) and Tinnitus is found in (40.0%) of patients. The mean pre- and post-operative outcomes in terms Pure tone (p<0.05), Air-bone gap (p<0.05) and Hearing gain (p<0.05). Conclusions: The present study concluded that Tympanoplasty is a beneficial procedure for hearing improvement and the eradication of the disease. Keywords: ENT

Comparative Study of Autologous Ossicular Graft versus Titanium Prosthesis (TORP & PORP) in Ossiculoplasty

Introduction This prospective cohort study aims to analyze and compare the outcomes of ossiculoplasty in terms of hearing results, intra and post operative course, using autograft incus and titanium middle ear prosthesis. Materials and Methods Patients with a history of chronic ear discharge and conductive deafness were included in the study. Out of 21 patients with ossicular chain defect included in the study, 10 patients underwent ossiculoplasty with autograft incus and 11 with titanium prosthesis. Pure tone audiogram was done after three months. Result In patients with incus ossiculoplasty, average Post operative PTA was 43.5 dB ± 7.934SD and Net gain in hearing was 10.7 decibels ± 15.478SD. In patients with titanium prosthesis ossiculoplasty, average Post operative PTA was 41.4 dB ± 4.789SD and Net gain in hearing was 16 decibels ± 11.981SD. Discussion Three patients underwent only Tympanoplasty post operative PTA was 42 ± 5.292 dB (incus- 39 dB, titanium- 48dB) and hearing gain was 13.33 ± 12.583 (incus-20dB, titanium-0dB). Five patients underwent intact canal wall with Tympanoplasty: Post operative PTA was 35 ± 3 dB (incus-35dB, titanium-30dB) and hearing gain was 23 ± 15.379 (incus-18.33dB, titanium-30dB). Thirteen patients underwent canal wall down procedure with Tympanoplasty: Post operative PTA was 45.67 ± 5.228 dB (incus-50.4dB, titanium-41.8dB) and hearing gain was 9.33 ± 12.309 (incus-2.4dB, titanium-14.285dB). Complications in the short period studied were nil in both groups. Conclusion Incus and titanium have equal postoperative hearing but in terms of hearing gain Titanium prosthesis gave a better hearing gain than incus.

Clinical Presentation of Cholesteatoma- A Study of 50 Cases

Introduction: Cholesteatoma is an abnormal accumulation of keratin-producing squamous epithelium in the middle ear, epitympanum, mastoid or petrous apex. It is a threedimensional epidermoid structure exhibiting independent growth, replacing middle ear mucosa, resorbing and replacing underlying bone. Although it is not a neoplastic lesion, it can be insidious and potentially dangerous to the patient. Objective: To find out the socio-demographic pattern, clinical presentation and complication of cholesteatoma. Materials and Methods: This prospective study of 50 cases was done in the Department of Otolaryngology and Head-Neck surgery, CMH, Dhaka adopting simple random sampling technique from July 2008 to Dec 2009. Data were collected by personal interview and clinical examination in a pre-designed data sheet and were analyzed by SPSS 20. Results: In this study, the highest number of patients (44%) was in the age group 11-20 years with a mean age of 17.2 years. Male were more affected (70%) and the male to female ratio was 2.33:1. Commonest symptom was otorrhoea (100%), followed by hearing impairment (80%), otalgia (16%), postauricular painful swelling (12%) and postauricular discharging sinus (10%). Extracranial complications were in 26% and intracranial complications in 12% of patients in this series. Abscess (temporal lobe + extra dural) were found in 02 cases (10%) by CT scan. A maximum number of patients had moderate conductive deafness (74.47%) followed by mild conductive deafness (19.15%) and only a few with severely mixed deafness (6.38%). Conclusion: Clinical presentation of cholesteatoma in this study was discharge and deafness. The discharge was thick purulent foul smelling, scanty, occasionally blood-stained. Grave intracranial complications in children developed because of illiteracy, poverty, lack of awareness and medical facilities mostly in the rural areas. So early diagnosis and prompt treatment was a necessity. Journal of Armed Forces Medical College Bangladesh Vol.13(1) 2017: 66-70