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Abstract
We report the case of an 18-year-old male patient who presented to us with unilateral conductive hearing loss with duplication of the lobule. An exploratory tympanotomy revealed stapes footplate fixation. A stapedotomy with insertion of a Teflon piston was performed with improvement in his hearing. We believe this is the first reported case of such an abnormality.
Key words: Stapes; External Ear; Surgery; Hearing Loss, Conductive
Introduction
Congenital anomalies of the middle ear are rare, sporadic and mostly non-familial. However these anomalies may present as a part of a syndrome such as Pfeiffer's, brancho-oto-renal, cervico-oculoacoustic, proximal symphalangia, Kleippel-Feil, frontometaphyseal dysplasia, or first and second branchial arch syndromes.12
Duplication of a part or whole of the pinna is also a rare anomaly, with a few cases reported in the literature.3 Again, such lesions may be sporadic or may arise as a part of a congenital syndrome. In our search of the medical literature, we did not find any case report of a congenital footplate fixation associated with duplication of the lobule, and the extreme rarity of such a case has prompted us to present this report.
Case report
An 18-year-old male patient presented to us with a nonprogressive hearing loss and a mass behind the right ear. The mass had been present since birth and the hearing loss had been noticeable since childhood. The patient gave a history of two to three episodes of otalgia and otorrhoea involving both ears in childhood: in each incidence this responded to medication. There was no history of any otological abnormalities in the family.
On examination, a well-formed duplicated lobule was present in the right post-aural region (Figures 1 and 2). The tympanic membranes were normal in appearance but tuning fork tests revealed a conductive hearing loss in the right ear and a pure tone audiogram revealed a conductive loss in the right ear with a mean air-bone gap of 45 dB in the three frequencies 256...