Skip to main content

Table 5 Olfaction, verbal IQ, neuroimaging, and audiology testing findings

From: Phenotypic characteristics of early Wolfram syndrome

Subject Test of olfaction Verbal IQ range Clinical neuroimaging findings Hearing loss Type of hearing loss
WU.WOLF-01 Total Anosmia ND ND Yes Sensorineural
WU.WOLF-02 Mild Microsmia Very Superior Normal No Normal
WU.WOLF-03 Total Anosmia High Average No pituitary bright spot Yes—hearing aid at age 6 Sensorineural
WU.WOLF-04 Severe Microsmia Average ND Yes—hearing aid at age 12 Sensorineural
WU.WOLF-05 Normosmia Borderline ND Yes—cochlear implant at age 2 Sensorineural
WU.WOLF-07 Normosmia High Average Loss of pituitary bright spot Yes, on right only—diagnosed at clinic Conductive
WU.WOLF-09 Mild Microsmia High Average Elevated T2 in the pons No Normal
WU.WOLF-10 Normosmia Average Several areas of T2 elevation No Normal
WU.WOLF-11 Total Anosmia High Average Normal Yes—diagnosed at clinic Mixed
WU.WOLF-12 Total Anosmia Average Several small T2 anomalies Yes—hearing aid at age 7 Sensorineural
WU.WOLF-13 Total Anosmia Average Small posterior pituitary Yes—diagnosed at clinic; no hearing aids Sensorineural
WU.WOLF-14 Normosmia High Average Elevated T2 in the periventricular occipital lobe Yes—hearing aids Sensorineural
WU.WOLF-15 Total Anosmia Superior Faint pituitary bright spot Yes—No hearing aids Sensorineural
WOLF2011-16 Moderate Microsmia High Average Multiple T2 abnormalities, punctate in all lobes. Poorly seen bright pituitary focus, either small or absent. Yes—No hearing aids Sensorineural
WU.WOLF-17 Mild Microsmia High Average Multiple punctate T2 foci in frontal lobes. Pituitary bright spot not seen Yes—No hearing aids Sensorineural
WU.WOLF-18 Mild Microsmia Very Superior Absent pituitary bright spot Yes—hearing aids Sensorineural
WU.WOLF-19 Mild Microsmia ND ND Yes—No hearing aids Sensorineural
WU.WOLF-22 Normosmia Average Several small foci of elevated flair signal Yes—Cochlear Implants Sensorineural
  1. ND = testing not done.