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If untreated, the disorder can result in a range of neurological and cutaneous symptoms, including sensorineural deficits and deafness. To understand early mechanistic abnormalities that may precede more generalized and nonspecific effects of metabolic deficits such as weight loss and acidosis, we have analyzed auditory brainstem responses (ABRs) in biotinidase\u2010deficient knockout (<jats:italic>Btd<\/jats:italic><jats:sup><jats:italic>\u2212\/\u2212<\/jats:italic><\/jats:sup>) mice in the periweaning period with or without dietary biotin supplementation. We find significant increases in the latency of wave V of the ABR elicited by pure tone stimuli at one octave intervals, which precede substantial increases in ABR thresholds. Finer interpeak latency analyses of these changes indicate they are confined to the latter ABR waves associated with the CNS and likely reflect slowed brainstem transmission time. In contrast, peripheral nervous system conduction velocity appears normal. Further, we find that biotin\u2010supplementation after the onset of symptoms reverses the latency shifts, which has significant relevance for early treatment in patients. Finally, ABR latencies in<jats:italic>Btd<\/jats:italic><jats:sup><jats:italic>\u2212\/\u2212<\/jats:italic><\/jats:sup>mice fed a biotin\u2010supplemented diet for the first month of life appear refractory to transmission time slowing during a subsequent bout of biotin deficiency. These data suggest a transient vulnerability window for biotin deficiency in the auditory brainstem. Finally, we also observe a developmental vulnerability window involving follicular melanosome production or melanocyte survival. Sensorineural deafness precedes peripheral hearing loss in developmental biotinidase deficiency and is transient if rescued by dietary biotin within a short developmental window.<\/jats:p>","DOI":"10.1007\/s10545-017-0049-z","type":"journal-article","created":{"date-parts":[[2017,5,17]],"date-time":"2017-05-17T09:02:02Z","timestamp":1495011722000},"page":"733-744","update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":5,"title":["Developmental window of sensorineural deafness in biotinidase\u2010deficient mice"],"prefix":"10.1002","volume":"40","author":[{"given":"Kathleen June","family":"Maheras","sequence":"first","affiliation":[{"name":"Center for Molecular Medicine and Genetics Wayne State University School of Medicine 3216 Scott Hall, 540 E Canfield Ave Detroit MI 48201 USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Kirit","family":"Pindolia","sequence":"additional","affiliation":[{"name":"Center for Molecular Medicine and Genetics Wayne State University School of Medicine 3216 Scott Hall, 540 E Canfield Ave Detroit MI 48201 USA"},{"name":"Department of Research Administration Henry Ford Hospital Detroit MI 48202 USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Barry","family":"Wolf","sequence":"additional","affiliation":[{"name":"Center for Molecular Medicine and Genetics Wayne State University School of Medicine 3216 Scott Hall, 540 E Canfield Ave Detroit MI 48201 USA"},{"name":"Department of Research Administration Henry Ford Hospital Detroit MI 48202 USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-7446-8990","authenticated-orcid":false,"given":"Alexander","family":"Gow","sequence":"additional","affiliation":[{"name":"Center for Molecular Medicine and Genetics Wayne State University School of Medicine 3216 Scott Hall, 540 E Canfield Ave Detroit MI 48201 USA"},{"name":"Carman and Ann Adams Department of Pediatrics Detroit MI 48201 USA"},{"name":"Department of Neurology Wayne State University School of Medicine Detroit MI 48201 USA"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2017,5,17]]},"reference":[{"key":"e_1_2_8_2_1","doi-asserted-by":"publisher","DOI":"10.3109\/00206098709081550"},{"key":"e_1_2_8_3_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00405-002-0480-z"},{"key":"e_1_2_8_4_1","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.91.24.11709"},{"key":"e_1_2_8_5_1","doi-asserted-by":"crossref","first-page":"211","DOI":"10.1111\/j.2517-6161.1964.tb00553.x","article-title":"An analysis of transformations","volume":"26","author":"Box GEP","year":"1964","journal-title":"J Royal Stat Soc, Series B"},{"key":"e_1_2_8_6_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.heares.2005.06.013"},{"key":"e_1_2_8_7_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.bpj.2015.08.012"},{"key":"e_1_2_8_8_1","doi-asserted-by":"publisher","DOI":"10.1083\/jcb.200808034"},{"key":"e_1_2_8_9_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.ymgme.2015.06.004"},{"key":"e_1_2_8_10_1","doi-asserted-by":"publisher","DOI":"10.1016\/0013-4694(79)90164-0"},{"volume-title":"An R companion to applied regression","year":"2011","author":"Fox J","key":"e_1_2_8_11_1"},{"key":"e_1_2_8_12_1","doi-asserted-by":"publisher","DOI":"10.1017\/S1740925X09990391"},{"key":"e_1_2_8_13_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0092-8674(00)81553-6"},{"key":"e_1_2_8_14_1","doi-asserted-by":"publisher","DOI":"10.1523\/JNEUROSCI.1640-04.2004"},{"key":"e_1_2_8_15_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0378-5955(02)00609-3"},{"key":"e_1_2_8_16_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.ymgme.2013.08.018"},{"volume-title":"R in action. 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