title: Cochlear implantation in pontine tegmental cap dysplasia.
abstract: Pontine tegmental cap dysplasia (PTCD) is an exceptionally rare brain stem and cerebellar malformation characterized by ventral pontine hypoplasia, vaulted pontine tegmentum, hypoplasia of the vermis, subtotal absence of middle cerebellar peduncles, lateralized course of the superior cerebellar peduncles, and absence or alteration of the inferior olivary nucleus. The main clinical features are multiple cranial neurophaties and ataxia. Sensorineural hearing loss of varying severity is almost always present. To date, 14 cases of PTCD have been reported in the literature. We present a child with PTCD and profound bilateral sensorineural hearing loss who underwent cochlear implantation. To the best of our knowledge, cochlear implantation in PTCD has not been previously reported. Functional outcome was assessed using the Speech Perception Categories and the Speech Intelligibility Rating scale. At 22 months' postoperative evaluation, the patient who was placed into speech perception category 0 (no detection of speech) preoperatively progressed to category 3 (beginning word identification). Before implantation, the child had connected speech unintelligible. At the last follow-up, she had connected speech intelligible to a listener who has little experience of a deaf person's speech. Cochlear implantation allowed this child to improve her quality of life, increasing her self-confidence, independence, and social integration.
authors.affiliation: Department of Otolaryngology, University of Parma, Via Gramsci 14, 43100 Parma, Italy. andreabacciu@yahoo.it
authors.forename: Andrea
authors.initials: A
authors.lastname: Bacciu
authors.forename: Francesca
authors.initials: F
authors.lastname: Ormitti
authors.forename: Enrico
authors.initials: E
authors.lastname: Pasanisi
authors.forename: Vincenzo
authors.initials: V
authors.lastname: Vincenti
authors.forename: Diego
authors.initials: D
authors.lastname: Zanetti
authors.forename: Salvatore
authors.initials: S
authors.lastname: Bacciu
mesh_terms: D001299:Audiometry; D002648:Child; D019929:Cochlear Implantation / Q000379:methods*; D003054:Cochlear Implants; D004569:Electroencephalography / Q000379:methods; D005260:Female; D005500:Follow-Up Studies; D006319:Hearing Loss, Sensorineural / Q000175:diagnosis / Q000209:etiology* / Q000601:surgery*; D006801:Humans; D008279:Magnetic Resonance Imaging / Q000379:methods; D009421:Nervous System Malformations / Q000175:diagnosis; D011149:Pons / Q000002:abnormalities*; D011788:Quality of Life; D013067:Speech Perception; D014057:Tomography, X-Ray Computed / Q000379:methods; D016896:Treatment Outcome