Phenotypes associated with this allele

• Allele Symbol: Otog^tm1Prs
• Allele Name: targeted mutation 1, Marie-Christine Simmler
• Allele ID: MGI:2181633
• Summary: 1 genotype

Jump to	Allelic Composition	Genetic Background	Genotype ID
hm1	Otog^tm1Prs/Otog^tm1Prs	129S2/SvPas-Otog^tm1Prs	MGI:3663256

Genotype
MGI:3663256

hm1

• Allelic Composition: Otog^tm1Prs/Otog^tm1Prs
• Genetic Background: 129S2/SvPas-Otog^tm1Prs

• ♀: phenotype observed in females
• ♂: phenotype observed in males
• N: normal phenotype

hearing/vestibular/ear

abnormal cochlea morphology ( J:60193 )

abnormal tectorial membrane morphology ( J:60193 )

• at P90, severely affected homozygotes exhibit ultrastructural defects in the TM fibrillar network organization that affect the resistance of TM to sound stimuli

abnormal semicircular canal morphology ( J:60193 )

abnormal crista ampullaris morphology ( J:60193 )

• in the semicircular canals, the cupula remains in contact with the neuroepithelium of the crista ampullaris until P30

• however, in older mice (P70-P90), the cupula is detached from the neuroepithelium

abnormal utricle morphology ( J:60193 )

abnormal vestibular saccule morphology ( J:60193 )

detached otolithic membrane ( J:60193 )

• starting at P2, otoconial membranes appear to be detached from the neuroepithelia in the utricular and saccular sensory regions, and are either sticking to the roof of these structures or floating in the endolymph

• however, the number and shape of otoconia remains intact

increased or absent threshold for auditory brainstem response ( J:60193 )

• overall, homozygotes respond to clicks at mean theshold levels of 8715 dB SPL relative to wild-type (354 dB SPL) and heterozygous (323.5 dB SPL) mice at frequencies ranging from 8 to 32 kHz

impaired hearing ( J:60193 )

• at 12-14 weeks of age, 6 of 18 homozygotes exhibit severe or profound bilateral hearing loss, while 12 show different degrees of hearing loss between the two ears, ranging from mild to profound

behavior/neurological

absent pinna reflex ( J:60193 )

• homozygotes display either absence of a Preyer's reflex at 3-4 weeks or become progressively unresponsive between 8 and 14 weeks

impaired righting response ( J:60193 )

• as early as P4, homozygotes show signs of vestibular dysfunction: when placed on their backs, they stay supine without trying to rotate to an upright position

impaired balance ( J:60193 )

• when dropped onto a soft surface, mutants fall on their backs, a behavior indicating a saccular defect

impaired swimming ( J:60193 )

• homozygotes fail to swim properly

abnormal posture ( J:60193 )

• at P20, homozygotes display loss of postural control

head tilt ( J:60193 )

• at P20, homozygotes display tilted heads

circling ( J:60193 )

• when swimming, all homozygotes adopt an underwater circling behavior