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The Journal of International Advanced... Aug 2019The aim of this report is to provide international recommendations for functional ear reconstruction in patients with microtia and aural atresia. All patients with...
The aim of this report is to provide international recommendations for functional ear reconstruction in patients with microtia and aural atresia. All patients with microtia and external auditory atresia should be seen in the setting of a multidisciplinary team and agreed treatment outcomes should be measured, so that techniques, approaches, and results can be compared. The methods are expert opinion from the members of the International Microtia and Atresia Workgroup (IMAW). The consensus recommendations reported herein take into account the variability in practice patterns present among experts in the field; the degree of consensus was quantified by presenting the percentage of above authors who agree or partially agree with each statement. Recommendations include the definition and classification of microtia/atresia, treatment of microtia, treatment of congenital aural atresia, flowchart of functional ear reconstruction, and future research directions. Patients with microtia and aural atresia can be guided by the consensus recommendations provided herein.
Topics: Child; Child, Preschool; Congenital Abnormalities; Congenital Microtia; Constriction, Pathologic; Ear; Ear, External; Ear, Middle; Humans; Plastic Surgery Procedures; Treatment Outcome
PubMed: 31418720
DOI: 10.5152/iao.2019.7383 -
Balkan Medical Journal Sep 2017Morphologically congenital sensorineural hearing loss can be investigated under two categories. The majority of congenital hearing loss causes (80%) are membranous...
Morphologically congenital sensorineural hearing loss can be investigated under two categories. The majority of congenital hearing loss causes (80%) are membranous malformations. Here, the pathology involves inner ear hair cells. There is no gross bony abnormality and, therefore, in these cases high-resolution computerized tomography and magnetic resonance imaging of the temporal bone reveal normal findings. The remaining 20% have various malformations involving the bony labyrinth and, therefore, can be radiologically demonstrated by computerized tomography and magnetic resonance imaging. The latter group involves surgical challenges as well as problems in decision-making. Some cases may be managed by a hearing aid, others need cochlear implantation, and some cases are candidates for an auditory brainstem implantation (ABI). During cochlear implantation, there may be facial nerve abnormalities, cerebrospinal fluid leakage, electrode misplacement or difficulty in finding the cochlea itself. During surgery for inner ear malformations, the surgeon must be ready to modify the surgical approach or choose special electrodes for surgery. In the present review article, inner ear malformations are classified according to the differences observed in the cochlea. Hearing and language outcomes after various implantation methods are closely related to the status of the cochlear nerve, and a practical classification of the cochlear nerve deficiency is also provided.
Topics: Classification; Cochlea; Cochlear Nerve; Ear, Inner; Hearing Loss, Sensorineural; Humans; Osteogenesis; Temporal Bone; Tomography, X-Ray Computed
PubMed: 28840850
DOI: 10.4274/balkanmedj.2017.0367 -
Journal of Anatomy Feb 2016
Topics: Animals; Ear; Mammals
PubMed: 26781524
DOI: 10.1111/joa.12431 -
Acta Oto-laryngologica Feb 2022Ear canal and middle ear tumors are rare and exhibit variability in histology and clinical manifestation. Surgical resection remains the treatment of choice, but...
BACKGROUND
Ear canal and middle ear tumors are rare and exhibit variability in histology and clinical manifestation. Surgical resection remains the treatment of choice, but individualized approach is needed to preserve function when possible.
AIMS/OBJECTIVES
To review the management and outcome of ear canal and middle ear tumors at an academic referral center.
MATERIALS AND METHODS
Helsinki University Hospital (HUS) patient files were searched for clinically and histologically confirmed ear canal and middle ear tumors over a 14-year period. The minimum follow-up time was 2 years.
RESULTS
Eighty-seven patients with 88 tumors were identified. There were 20 (23%) benign external auditory canal (EAC), 36 (41%) benign middle ear space (MES), 29 (33%) malignant EAC, and 3 (3%) malignant MES tumors. Most (92%) tumors were managed with primary resection. Thirty-five percent of the operatively managed patients had a residual or a recurrent tumor.
CONCLUSIONS AND SIGNIFICANCE
EAC and MES tumors show great diagnostic and histologic heterogeneity with need for individualized investigative and treatment approaches. In benign tumors, we advocate aggressive local surgical control without sacrificing vital structures. In malignant tumors, we recommend local surgical control with or without adjunct RT.
Topics: Ear Canal; Ear Neoplasms; Ear, Middle; Humans; Neoplasm Recurrence, Local; Retrospective Studies
PubMed: 35148225
DOI: 10.1080/00016489.2022.2032824 -
Head and Neck Pathology Sep 2018The temporal bone is one of the more complex structures at the skull base that houses the hearing and vestibular organs, numerous nerves, and vessels. A host of... (Review)
Review
The temporal bone is one of the more complex structures at the skull base that houses the hearing and vestibular organs, numerous nerves, and vessels. A host of inflammatory and neoplastic processes can occur within the temporal bone that often necessitate permanent and frozen section pathologic examination. A number of simple to complex surgical procedures are used to manage temporal bone pathology. This chapter will provide a brief overview of normal temporal bone anatomy, common surgical approaches, normal histology, and indications for pathologic examination.
Topics: Ear; Humans; Otorhinolaryngologic Surgical Procedures; Temporal Bone
PubMed: 30069845
DOI: 10.1007/s12105-018-0926-2 -
Physiological Research May 2018This review is focused on the unusual composition of the endolymph of the inner ear and its function in mechanoelectrical transduction. The role of K(+) and Ca(2+) in... (Review)
Review
This review is focused on the unusual composition of the endolymph of the inner ear and its function in mechanoelectrical transduction. The role of K(+) and Ca(2+) in excitatory influx, the very low Na(+), Ca(2+) and Mg(2+) concentrations of endolymph, stereocilia structure of hair cells and some proteins involved in mechanosensory signal transduction with emphasis on auditory receptors are presented and analyzed in more details. An alternative hypothetical model of ciliary structure and endolymph with a 'normal' composition is discussed. It is concluded that the unique endolymph cation content is more than an energy saving mechanism that avoids disturbing circulatory vibrations to achieve a much better mechanosensory resolution. It is the only possible way to fulfil the requirements for a precise ciliary mechanoelectrical transduction in conditions where pressure events with quite diverse amplitudes and duration are transformed into adequate hair cell membrane depolarizations, which are regulated by a sensitive Ca(2+)-dependent feedback tuning.
Topics: Animals; Ear, Inner; Endolymph; Hair Cells, Auditory, Inner; Humans; Mechanotransduction, Cellular
PubMed: 29303598
DOI: 10.33549/physiolres.933684 -
Purinergic Signalling Jun 2022The inner ear comprises the cochlea and vestibular system, which detect sound and acceleration stimulation, respectively. The function of the inner ear is regulated by... (Review)
Review
The inner ear comprises the cochlea and vestibular system, which detect sound and acceleration stimulation, respectively. The function of the inner ear is regulated by ion transport activity among sensory epithelial cells, neuronal cells, non-sensory epithelial cells, and luminal fluid with a unique ionic composition of high [K] and low [Na], which enables normal hearing and balance maintenance. One of the important mechanisms regulating ion transport in the inner ear is purinergic signaling. Various purinergic receptors are distributed throughout inner ear epithelial cells and neuronal cells. To date, most studies have focused on the role of purinergic receptors in the cochlea, and few studies have examined these receptors in the vestibular system. As purinergic receptors play an important role in the cochlea, they would likely do the same in the vestibular system, which is fairly similar to the cochlea in cellular structure and function. Based on available studies performed to date, purinergic signaling is postulated to be involved in the regulation of ion homeostasis, protection of hair cells, otoconia formation, and regulation of electrical signaling from the sensory epithelium to vestibular neurons. In this review, the distribution and roles of purinergic receptors in the peripheral vestibular system are summarized and discussed.
Topics: Cochlea; Ear, Inner; Receptors, Purinergic; Signal Transduction; Vestibular System
PubMed: 35344126
DOI: 10.1007/s11302-022-09855-5 -
The Journal of the Acoustical Society... Apr 2021The mouse is an important animal model for hearing science. However, our knowledge of the relationship between mouse middle-ear (ME) anatomy and function is limited. The...
The mouse is an important animal model for hearing science. However, our knowledge of the relationship between mouse middle-ear (ME) anatomy and function is limited. The ME not only transmits sound to the cochlea in the forward direction, it also transmits otoacoustic emissions generated in the cochlea to the ear canal (EC) in the reverse direction. Due to experimental limitations, a complete characterization of the mouse ME has not been possible. A fully coupled finite-element model of the mouse EC, ME, and cochlea was developed and calibrated against experimental measurements. Impedances of the EC, ME, and cochlea were calculated, alongside pressure transfer functions for the forward, reverse, and round-trip directions. The effects on sound transmission of anatomical changes such as removing the ME cavity, pars flaccida, and mallear orbicular apophysis were also calculated. Surprisingly, below 10 kHz, the ME cavity, eardrum, and stapes annular ligament were found to significantly affect the cochlear input impedance, which is a result of acoustic coupling through the round window. The orbicular apophysis increases the delay of the transmission line formed by the flexible malleus, incus, and stapes, and improves the forward sound-transmission characteristics in the frequency region of 7-30 kHz.
Topics: Acoustic Stimulation; Acoustics; Animals; Cochlea; Ear, Middle; Mice; Round Window, Ear; Sound; Stapes
PubMed: 33940924
DOI: 10.1121/10.0004218 -
Expert Opinion on Biological Therapy Feb 2019Sound is integral to communication and connects us to the world through speech and music. Cochlear hair cells are essential for converting sounds into neural impulses.... (Review)
Review
INTRODUCTION
Sound is integral to communication and connects us to the world through speech and music. Cochlear hair cells are essential for converting sounds into neural impulses. However, these cells are highly susceptible to damage from an array of factors, resulting in degeneration and ultimately irreversible hearing loss in humans. Since the discovery of hair cell regeneration in birds, there have been tremendous efforts to identify therapies that could promote hair cell regeneration in mammals.
AREAS COVERED
Here, we will review recent studies describing spontaneous hair cell regeneration and direct cellular reprograming as well as other factors that mediate mammalian hair cell regeneration.
EXPERT OPINION
Numerous combinatorial approaches have successfully reprogrammed non-sensory supporting cells to form hair cells, albeit with limited efficacy and maturation. Studies on epigenetic regulation and transcriptional network of hair cell progenitors may accelerate discovery of more promising reprogramming regimens.
Topics: Animals; Cellular Reprogramming; Ear, Inner; Epigenesis, Genetic; Hair Cells, Auditory; Humans; Regeneration
PubMed: 30584811
DOI: 10.1080/14712598.2019.1564035 -
European Annals of Otorhinolaryngology,... Mar 2022
Topics: Ear Auricle; Ear, External; Humans
PubMed: 34417141
DOI: 10.1016/j.anorl.2021.05.021