{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,12]],"date-time":"2026-10-12T17:01:00Z","timestamp":1791824460790,"version":"4.3.4"},"reference-count":20,"publisher":"Cambridge University Press (CUP)","issue":"5","license":[{"start":{"date-parts":[[2015,5,21]],"date-time":"2015-05-21T00:00:00Z","timestamp":1432166400000},"content-version":"unspecified","delay-in-days":20,"URL":"https:\/\/www.cambridge.org\/core\/terms"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["J. Laryngol. Otol."],"published-print":{"date-parts":[[2015,5]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec id=\"S0022215114003375_sec1\"><jats:title>Background:<\/jats:title><jats:p>Brown\u2013Vialetto\u2013Van Laere syndrome is a rare neurological disorder characterised by pontobulbar palsy and sensorineural hearing loss. Hearing rehabilitation continues to be a challenge because the exact lesion site is unknown.<\/jats:p><\/jats:sec><jats:sec id=\"S0022215114003375_sec2\"><jats:title>Case report:<\/jats:title><jats:p>We examined the clinical and audiological profiles of a case series comprising four siblings with Brown\u2013Vialetto\u2013Van Laere syndrome who had decreased hearing and poor speech discrimination. Audiological investigations revealed normal otoacoustic emissions with absent auditory brainstem responses and middle-ear reflexes in sensorineural hearing loss, suggestive of auditory neuropathy spectrum disorder.<\/jats:p><\/jats:sec><jats:sec id=\"S0022215114003375_sec3\"><jats:title>Conclusion:<\/jats:title><jats:p>The sensorineural hearing loss in Brown\u2013Vialetto\u2013Van Laere syndrome patients is a retrocochlear pathology resembling auditory neuropathy spectrum disorder, with the lesion being most probably of post-synaptic origin. Early cochlear implantation along with high-dose riboflavin represents a possible rehabilitation therapy. However, further research is needed to confirm this. This report emphasises the need for a thorough neurological evaluation of auditory neuropathy spectrum disorder patients.<\/jats:p><\/jats:sec>","DOI":"10.1017\/s0022215114003375","type":"journal-article","created":{"date-parts":[[2015,7,2]],"date-time":"2015-07-02T10:40:46Z","timestamp":1435833646000},"page":"504-508","source":"Crossref","is-referenced-by-count":10,"title":["Auditory neuropathy spectrum disorder with Brown\u2013Vialetto\u2013Van Laere syndrome: challenges in hearing rehabilitation"],"prefix":"10.1017","volume":"129","author":[{"given":"R","family":"Chandran","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"M","family":"Alexander","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"P","family":"Naina","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"A","family":"Balraj","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"56","published-online":{"date-parts":[[2015,5,21]]},"reference":[{"key":"S0022215114003375_ref7","doi-asserted-by":"publisher","DOI":"10.1093\/brain\/119.3.741"},{"key":"S0022215114003375_ref14","doi-asserted-by":"publisher","DOI":"10.1146\/annurev.physiol.61.1.477"},{"key":"S0022215114003375_ref13","doi-asserted-by":"publisher","DOI":"10.1007\/BF01800662"},{"key":"S0022215114003375_ref18","doi-asserted-by":"publisher","DOI":"10.1186\/gm212"},{"key":"S0022215114003375_ref17","doi-asserted-by":"publisher","DOI":"10.1177\/08830738060210060501"},{"key":"S0022215114003375_ref2","doi-asserted-by":"publisher","DOI":"10.1258\/0022215054273179"},{"key":"S0022215114003375_ref5","doi-asserted-by":"publisher","DOI":"10.1136\/jmg.27.3.176"},{"key":"S0022215114003375_ref3","doi-asserted-by":"publisher","DOI":"10.1017\/S0022215110001982"},{"key":"S0022215114003375_ref8","doi-asserted-by":"publisher","DOI":"10.1097\/WCO.0b013e32834f0351"},{"key":"S0022215114003375_ref16","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2990.1993.tb00409.x"},{"key":"S0022215114003375_ref12","doi-asserted-by":"publisher","DOI":"10.1007\/s10545-010-9242-z"},{"key":"S0022215114003375_ref20","doi-asserted-by":"publisher","DOI":"10.1152\/jn.00985.2004"},{"key":"S0022215114003375_ref9","doi-asserted-by":"publisher","DOI":"10.1097\/MAO.0b013e318164d0f6"},{"key":"S0022215114003375_ref10","doi-asserted-by":"publisher","DOI":"10.1016\/j.ajhg.2010.02.006"},{"key":"S0022215114003375_ref4","doi-asserted-by":"publisher","DOI":"10.1002\/(SICI)1096-8628(20000515)92:2<117::AID-AJMG7>3.0.CO;2-C"},{"key":"S0022215114003375_ref19","doi-asserted-by":"publisher","DOI":"10.1111\/j.1469-8749.2011.04142.x"},{"key":"S0022215114003375_ref1","first-page":"3","article-title":"Brown\u2013Vialetto\u2013Van Laere syndrome","volume":"17","author":"Sathasivam","year":"2008","journal-title":"Orphanet J Rare Dis"},{"key":"S0022215114003375_ref15","doi-asserted-by":"publisher","DOI":"10.1097\/MAO.0b013e318164d0f6"},{"key":"S0022215114003375_ref11","doi-asserted-by":"publisher","DOI":"10.1093\/jb\/mvn181"},{"key":"S0022215114003375_ref6","doi-asserted-by":"publisher","DOI":"10.1016\/j.nmd.2005.05.003"}],"container-title":["The Journal of Laryngology &amp; Otology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.cambridge.org\/core\/services\/aop-cambridge-core\/content\/view\/S0022215114003375","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2019,4,21]],"date-time":"2019-04-21T01:39:28Z","timestamp":1555810768000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.cambridge.org\/core\/product\/identifier\/S0022215114003375\/type\/journal_article"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2015,5]]},"references-count":20,"journal-issue":{"issue":"5","published-print":{"date-parts":[[2015,5]]}},"alternative-id":["S0022215114003375"],"URL":"https:\/\/doi.org\/10.1017\/s0022215114003375","relation":{},"ISSN":["0022-2151","1748-5460"],"issn-type":[{"value":"0022-2151","type":"print"},{"value":"1748-5460","type":"electronic"}],"subject":[],"published":{"date-parts":[[2015,5]]}}}