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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.