Congenital oval window aplasia: An unusual cause of conductive hearing loss in an adult

@#<p style="text-align: justify;"><strong>OBJECTIVES:</strong> To  report  a  case  of  congenital  oval  window  aplasia  (COWA)  in  a  Filipino  adult presenting   with      unilateral   maximal   conductive   hearing   loss   and   discuss   the   diagnostic considerations, pathophysiology and management.<br /><strong>METHODS:</strong><br /><strong>Design:</strong> Case Report<br /><strong>Setting:</strong> Tertiary Public Referral Center<br /><strong>Patient:</strong> One <br /><strong>RESULTS:</strong> Audiometric evaluation showed a maximal unilateral left conductive hearing loss. High resolution temporal bone CT showed absence of the oval window on the left along with facial and stapes  abnormalities.    Exploratory  tympanotomy  showed  an  aberrant  facial  nerve,  monopodal and  abnormally  located  stapes  and  absent  oval  window. Postoperative  hearing  gain  achieved after a neo-oval window and Schuknecht piston wire prosthesis remained stable over two years.<br /><strong>CONCLUSION:</strong> A congenital minor ear anomaly classified as Cremers Class 4a in which a congenital oval  window  aplasia  was  associated  with  an  aberrant  facial  nerve  anomaly  and  a  monopodal stapes is reported. Recent literature supported the view that congenital oval window aplasia can in selected cases be amenable to various surgical approaches and a stable postoperative hearing gain is achievable in the long term.</p>

Congenital Middle Ear Cholesteatoma in Children / 대한이비인후과학회지

BACKGROUND AND OBJECTIVES: Congenital middle ear cholesteatoma (CMEC) is a rare entity that may go undiagnosed for years. Aims of this study were to assess the characteristic features and recurrence of CMEC in pediatric patients of different stages and to determine the value of preoperative CT scan in CMEC. SUBJECTS AND METHOD: Thirty cases of CMEC under 15 years old that had been treated at the hospitals of the Catholic University from 1995 through 2005 were reviewed retrospectively. The age range was from 2 to 13 with the mean age of 6.2. The main outcome measures were CT findings, surgical findings, recurrence rate and hearing assessment. RESULTS: Preoperative CT scan accurately predicted the extent of the cholesteatoma seen during surgery in 25/30 (83.3%). The recurrence rate of CMEC was 6.7% (2/30) and all of recurrent cases were belonged to stage IV. In the recurrent cases, cholesteatomas were extended to sinus tympani and facial recess at revisional operation as well as at the initial operation. CONCLUSION: Preoperative CT scan is essential in defining the extent of existing pathology. The intra-operative CMEC extension and location influence the outcome of surgery. In the higher stages, careful eradication of disease, particularly in the region of sinus tympani and facial recess, are recommended.

Congenital Middle Ear Cholesteatoma / 대한이비인후과학회지

BACKGROUND AND OBJECTIVES: Congenital middle ear cholesteatoma (CMEC) is a keratinous mass behind an intact tympanic membrane. CMEC does not have a history of instrumentation and is less common than acquired one. Many theories have been put forward to explain the pathophysiology of CMEC, however, none of these so far have been convincingly proven. This clinical study was performed to investigate the characteristic features of CMEC and to evaluate the correlation between pathophysiology and CMEC by retrospectivly reviewing the cases. MATERIALS AND METHOD: The medical records of patients who underwent otologic procedures at the hospitals of the Catholic university from January 1993 to September 1998 have been reviewed. They were ten males and four females, ranging in age from 4 to 59 (mean age 18). RESULTS: Three of the 14 patients had the lesions isolated to the anterosuperior quadrant of the mesotympanum which were cystic, easily removed and did not affect hearing. The others had more serious condition with extension into the posterior mesotympanum, which were large, often too extensive to indicate a formative site, and causing ossicular damage. CONCLUSION: CMEC presents in two distinctive forms according to the site of formation: the anterosuperior and posterior mesotympanum. The review suggest that the pathophysiology of posterior lesions may be different from anterior ones. For early diagnosis of CMEC, screening program should be carried out in children to prevent the more extensive diseases.