Congenital oval or round window anomaly with or without abnormal facial nerve course:  surgical results for 15 ears.
OBJECTIVE
To describe the audiometric results in a consecutive series of patients with congenital ossicular aplasia (Class 4a) or dysplasia of the oval and/or round window (Class 4b), which might include a possible anomalous course of the facial nerve.


METHODS
Retrospective chart study.


METHODS
Tertiary referral center.


METHODS
A tertiary referral center study with a total of 14 patients with congenital minor ear anomalies as part of a consecutive series (n = 89) who underwent exploratory tympanotomies (15 ears).


METHODS
Audiometric results.


RESULTS
In 8 of 15 ears, ossicular reconstruction was attempted. In the short term (1 mo), there was a serviceable hearing outcome (air-bone gap closure to within 25 dB) in 4 ears. However, the long-term results showed deterioration because of an increased air-bone gap in all but 1 ear. No facial nerve lesion was observed postoperatively.


CONCLUSIONS
Congenital dysplasia or aplasia of the oval and/or round window is an uncommon congenital minor ear anomaly. Classical microsurgical opportunities are rare in this group of anomalies. Newer options for hearing rehabilitation, such as the osseointegrated passive bone conduction devices, have become viable alternatives for conventional air conduction hearing devices. In the near future, upcoming active bone conduction devices might become the most preferred surgical option. In cases in which the facial nerve is only partially overlying the oval window, a type of malleostapedotomy procedure might result in a serviceable postoperative hearing level.
Donders Centre for Brain, Cognition and Behavior, Centre for Clinical Neuroscience, Radboud University Nijmegen Medical Centre, Nijmegen, The Netherlands. H.Thomeer@kno.umcn.nl
Henricus
H
Thomeer
Henricus
H
Kunst
Berit
B
Verbist
Cor
C
Cremers
D000293:Adolescent; D000328:Adult; D001299:Audiometry; D002648:Child; D004429:Ear Ossicles / Q000002:abnormalities / Q000503:physiopathology / Q000601:surgery*; D005154:Facial Nerve / Q000002:abnormalities / Q000503:physiopathology / Q000601:surgery*; D006314:Hearing Loss, Conductive / Q000151:congenital / Q000503:physiopathology / Q000601:surgery*; D006801:Humans; D008297:Male; D008875:Middle Aged; D010046:Oval Window, Ear / Q000002:abnormalities / Q000503:physiopathology / Q000601:surgery*; D012189:Retrospective Studies; D012405:Round Window, Ear / Q000002:abnormalities* / Q000503:physiopathology / Q000601:surgery*; D016896:Treatment Outcome