caa a22 4500 388084278 CHVBK 20180307125226.0 cr unu---uuuuu 161130e199908 xx s 000 0 eng 10.1017/S0022215100145001 doi S0022215100145001 pii (NATIONALLICENCE)cambridge-10.1017/S0022215100145001 Useful residual hearing despite radiological findings suggestive of anacusis [Elektronische Daten] A severe malformation of the inner ear, often referred to as severe labyrinthine dysplasia or common cavity deformity, consists of an absent or dilated cochlear basal coil, wide communication with the vestibule and a tapered internal acoustic meatus and can be associated with absent hearing. We discuss two children with severe labyringthine dysplasia as shown by computed tomography (CT) scans and, in the first case, an absent VIIIth nerve bilaterally shown by magnetic resonance imaging (MRI). In 1995, both cases were precluded from cochlear implantation, on the basis of the absent VIIIth nerve (first case) and increased risk of CSF leak during operation (second case). However, audiometric results and vocalization patterns of both children suggested the presence of some residual hearing function, while recently reported specific surgical techniques have been found to be safe and effective in the cochlear implantation of the common cavity deformity. The management of such cases should be decided on the grounds of a full audiological assessment in conjunction with the radiological features, in the light of current surgical trends shown to be safe and effective. Copyright © JLO (1984) Limited 1999 Labyrinth nationallicence Dysplasia nationallicence Cochlear implant nationallicence Bamiou Doris Eva Audiology Department, Great Ormond Street Hospital, London, UK. Mahoney Cliodna O. Audiology Department, Great Ormond Street Hospital, London, UK. Sirimanna Tony Audiology Department, Great Ormond Street Hospital, London, UK. The Journal of Laryngology & Otology Cambridge University Press 113/8(1999-08), 714-716 0022-2151 113:8<714 1999 113 JLO https://doi.org/10.1017/S0022215100145001 text/html Onlinezugriff via DOI 1 research-article jats NATIONALLICENCE 856 40 https://doi.org/10.1017/S0022215100145001 text/html Onlinezugriff via DOI NATIONALLICENCE 700 1- Bamiou Doris Eva Audiology Department, Great Ormond Street Hospital, London, UK NATIONALLICENCE 700 1- Mahoney Cliodna O. Audiology Department, Great Ormond Street Hospital, London, UK NATIONALLICENCE 700 1- Sirimanna Tony Audiology Department, Great Ormond Street Hospital, London, UK NATIONALLICENCE 773 0- The Journal of Laryngology & Otology Cambridge University Press 113/8(1999-08), 714-716 0022-2151 113:8<714 1999 113 JLO CC0 http://creativecommons.org/publicdomain/zero/1.0 nationallicence BK010053 XK010053 XK010000 NATIONALLICENCE NATIONALLICENCE NL-cambridge