Dataset Information

Knockout of Pannexin-1 Induces Hearing Loss.

ABSTRACT: Mutations of gap junction connexin genes induce a high incidence of nonsyndromic hearing loss. Pannexin genes also encode gap junctional proteins in vertebrates. Recent studies demonstrated that Pannexin-1 (Panx1) deficiency in mice and mutation in humans are also associated with hearing loss. So far, several Panx1 knockout (KO) mouse lines were established. In general, these Panx1 KO mouse lines demonstrate consistent phenotypes in most aspects, including hearing loss. However, a recent study reported that a Panx1 KO mouse line, which was created by Genentech Inc., had no hearing loss as measured by the auditory brainstem response (ABR) threshold at low-frequency range (<24 kHz). Here, we used multiple auditory function tests and re-examined hearing function in the Genentech Panx1 (Gen-Panx1) KO mouse. We found that ABR thresholds in the Gen-Panx1 KO mouse were significantly increased, in particular, in the high-frequency region. Moreover, consistent with the increase in ABR threshold, distortion product otoacoustic emission (DPOAE) and cochlear microphonics (CM), which reflect active cochlear amplification and auditory receptor current, respectively, were significantly reduced. These data demonstrated that the Gen-Panx1 KO mouse has hearing loss and further confirmed that Panx1 deficiency can cause deafness.

SUBMITTER: Chen J

PROVIDER: S-EPMC5983795 | biostudies-literature | 2018 Apr

REPOSITORIES: biostudies-literature

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Knockout of Pannexin-1 Induces Hearing Loss.

Chen Jin J Liang Chun C Zong Liang L Zhu Yan Y Zhao Hong-Bo HB

International journal of molecular sciences 20180430 5

Mutations of gap junction connexin genes induce a high incidence of nonsyndromic hearing loss. Pannexin genes also encode gap junctional proteins in vertebrates. Recent studies demonstrated that Pannexin-1 (Panx1) deficiency in mice and mutation in humans are also associated with hearing loss. So far, several Panx1 knockout (KO) mouse lines were established. In general, these Panx1 KO mouse lines demonstrate consistent phenotypes in most aspects, including hearing loss. However, a recent study r ...[more]

PMID: 29710868

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Project description:OBJECTIVE:Hearing loss is the most common sensory deficit and congenital anomaly, yet the decision-making processes involved in disclosing hearing loss have been little studied. To address this issue, we have explored the phrases that adults with hearing loss use to disclose their hearing loss. DESIGN:Since self-disclosure research has not focused on hearing loss-specific issues, we created a 15-question survey about verbally disclosing hearing loss. English speaking adults (>18 years old) with hearing loss of any etiology were recruited from otology clinics in a major referral hospital. Three hundred and thirty-seven participants completed the survey instrument. Participants' phrase(s) used to tell people they have hearing loss were compared across objective characteristics (age; sex; type, degree, and laterality of hearing loss; word recognition scores) and self-reported characteristics (degree of hearing loss; age of onset and years lived with hearing loss; use of technology; hearing handicap score). RESULTS:Participants' responses revealed three strategies to address hearing loss: Multipurpose disclosure (phrases that disclose hearing loss and provide information to facilitate communication), Basic disclosure (phrases that disclose hearing loss through the term, a label, or details about the condition), or nondisclosure (phrases that do not disclose hearing loss). Variables were compared between patients who used and who did not use each disclosure strategy using ? or Wilcoxon rank sum tests. Multipurpose disclosers were mostly female (p = 0.002); had experienced reactions of help, support, and accommodation after disclosing (p = 0.008); and had experienced reactions of being overly helpful after disclosing (p=0.039). Basic disclosers were predominantly male (p = 0.004); reported feeling somewhat more comfortable disclosing their hearing loss over time (p = 0.009); had not experienced reactions of being treated unfairly or discriminated against (p = 0.021); and were diagnosed with mixed hearing loss (p = 0.004). Nondisclosers tended not to disclose in a group setting (p = 0.002) and were diagnosed with bilateral hearing loss (p = 0.005). In addition, all of the variables were examined to build logistic regression models to predict the use of each disclosure strategy. CONCLUSIONS:Our results reveal three simple strategies for verbally addressing hearing loss that can be used in a variety of contexts. We recommend educating people with hearing loss about these strategies-this could improve the experience of disclosing hearing loss, and could educate society at large about how to interact with those who have a hearing loss.

Dataset Information

Knockout of Pannexin-1 Induces Hearing Loss.

Publications

Knockout of Pannexin-1 Induces Hearing Loss.

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