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Hearing Impairment
       Sound waves are transmitted from the ear-  through the air is impaired, bone conduction
       drum (tympanum) via the ossicles to the fe-  remains normal (→ A).
       nestra vestibuli (vestibular window) (→ A).  The hair cells can be damaged by sound
       The transmitting apparatus in the middle ear  stress (i.e., impingement of too loud a sound
       acts as an impedance converter. Without it  for too long) and ischemia. But thanks to its
    Systems  98% of sound energy would be reflected away  high glycogen content they can survive short
       because of the markedly different resistances
                                       periods of ischemia by anaerobic glycolysis.
       to the sound waves in the air and in the fluid
                                       Hair cells can also be damaged by certain
                                       drugs—such as aminoglycoside antibiotics and
       of the inner ear. Invagination of the fenestra
    Neuromuscular and Sensory  of the fenestra cochleae (cochlear window).  via the stria vascularis, are accumulated in the
                                       the chemotherapeutic agent cisplatin—that,
       vestibuli results in simultaneous evagination
       The eardrum normally protects the latter
                                       endolymph. This results in inner ear hearing
       against external sound waves and conducts
                                       loss that affects air and bone conduction
       the sound energy specifically toward the fe-
                                       equally (→ B4). Both the hearing threshold
       nestra vestibuli. Sound waves can also be
                                       and the active component of basilar mem-
                                       brane displacement are affected, so that dis-
       transmitted to the bones of the skull and can
                                       crimination
       thus stimulate the inner ear. However, this re-
                                                         higher-frequency
                                                  different
                                               of
                                       depolarization of the inner hair cells can pro-
         The oscillation of the fenestra vestibuli pro-
       duces traveling waves in the inner ear, at first
                                       duce an unusual and disturbing sound sensa-
    10  quires a much greater energy of sound.  tones is impaired (→ B5). Lastly, inadequate
                                       tion (subjective tinnitus). This can also be
       spreading along the scala vestibuli. The stereo-
       cilia of the outer and inner hair cells are bent  caused by inadequate excitation of neurons in
       by evagination of the cochlear septum with  the auditory pathway or the auditory cortex.
       the basilar membrane and the organ of Corti  Stiffening of the basilar membrane disturbs
       at a frequency-dependent location (→ B1).  the micromechanics and thus probably con-
                         +
       This leads to the opening of K channels in the  tributes to hearing loss in the elderly (→ B1).
       cell membrane. The endolymph in which the  Inner ear deafness can also be the result of
       stereocilia of the hair cells are suspended  abnormal endolymph secretion. Thus loop
                       +
       (→ B2) has a very high K concentration (ca.  diuretics at high dosage not only inhibit renal
                                                         –
                 +
                                                      +
                                                    +
       150 mmol/L). K is secreted by the epithelial  but also auditory Na -K -2 Cl cotransport. In
                           +
                                 –
                             +
       cells of the stria vascularis, by Na -K -2 Cl co-  addition, there is a known (but rare) genetic
                                                     +
                    +
                      +
       transport and by Na /K -ATPase in the anti-  defect of the luminal K channel. The channel,
       luminal membrane, as well as by a luminal K +  which consists of two subunits (IsK/Kv LQT1 ), is
                         +
       channel (→ B3). When the K channels in the  also expressed in the heart (as well as other or-
                                 +
       membrane of the hair cells are opened, K en-  gans), where it participates in repolarization. A
       ters the cells and depolarizes them. This depo-  defect of Kv LQT1 or IsK results not only in deaf-
       larization then triggers the release of the  ness but also in delayed myocardial repolari-
       transmitter, especially in the inner hair cells.  zation (long QT interval [Jervell, Lange–Nielsen
       By contracting, the outer hair cells increase  syndrome]). Abnormal absorption of endo-
       the local traveling wave and thus the amount  lymph can also cause deafness. The endo-
       of stimulation of the hair cells.  lymph space becomes evaginated, distorting
         Causes of deafness. A tear in the eardrum, a  the relationship between hair cells and tectori-
       lesion in the ossicles, or immobilization of the  al membrane (endolymph edema; → B6). Fi-
       conduction apparatus, for example, caused by  nally, increased permeability between the en-
       a purulent middle ear infection, dampen  dolymph and perilymph spaces may be re-
       transmission to the fenestra vestibuli. Further-  sponsible for Ménière’s disease, which is char-
       more, if there is a hole in the drum, the fenes-  acterized by attacks of deafness and vertigo
       tra cochleae is no longer protected. This results  (→ B7).
  328  in middle ear hearing loss. While conduction
       Silbernagl/Lang, Color Atlas of Pathophysiology © 2000 Thieme
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