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. Author manuscript; available in PMC: 2023 Mar 1.
Published in final edited form as: Ear Hear. 2021 Sep 15;43(2):582–591. doi: 10.1097/AUD.0000000000001126

Table 6.

Average Hearing Aid Benefit in Patients with PBD-ZSD or DBPD Mutations

Characteristic Benefita
PEX1 - G843D/Null
 # Patients w. Unaided + Aided Responses 2
  Binaural/Ear unspecified 35 dB
  Binaural/Ear unspecified SAT/SRT 35– 45 dB
PEX1 - G843D/G843D
 # Patients w. Unaided + Aided Responses 6
  Binaural/Ear unspecified 46 – 61 dB
  Binaural/Ear unspecified SAT/SRT 30 – 50 dB
  Right Ear 26 – 46 dB
  Right Ear SAT/SRT 35 – 50 dB
  Left Ear 29 – 50 dB
  Left Ear SAT/SRT 35 – 50 dB
Other PEX - Intermediate & Mild
 # Patients w. Unaided + Aided Responses 3
  Right Ear 36 – 44 dB
  Right Ear SAT/SRT 50 dB
  Left Ear 35 – 44 dB
  Left Ear SAT/SRT 50 dB
DBPD
 # Patients w. Unaided + Aided Responses 2
  Binaural/Ear unspecified 31 – 35 dB
a

Data indicates range of group benefit with amplification in dB. Differences in unaided and aided 4F-PTAs and SAT/SRTs were calculated for the first test date with unaided and aided response to calculate individual benefits. These were then averaged to determine mean group benefits from amplification.