The Hearing of Erastus "Deaf" Smith, Hero of the Texas Revolution.

OBJECTIVES: To describe the speech and hearing abilities of Erastus "Deaf" Smith, the first Texas Ranger, and speculate on the etiology of his hearing loss. METHODS: Review of credible historical data of what Erastus could and could not hear, descriptions of his voice, and loudness tolerance. Modern analysis of acoustical physics data generated by 0.69-inch diameter non-spinning musket balls. RESULTS: The onset of Erastus' bilateral hearing loss was early childhood. He could hear some. He was intolerant of very loud sounds. His speech was "weak", "squeaky" and high-pitched. He did not use manual communication. He could not hear musket balls passing near his head. The frequency of sound generated by flying 0.69-inch diameter musket balls has been determined as predominantly in the range of 2000-2500 Hz. In contrast to most bilateral childhood sensorineural hearing losses that are worse in high and mid frequencies, Erastus' hearing loss was worse in the low and mid frequencies - preserving at least some high-frequency hearing. CONCLUSION: Erastus' reverse-slope hearing problem may well have been attributable to incomplete partition type III. If you can't hear it, you can't speak it. LEVEL OF EVIDENCE: NA Laryngoscope, 134:3532-3536, 2024.

Malleus and incus: correlates of size.

OBJECTIVES: Wide ranges of dimensions of the malleus and incus have been reported for various human populations. Unaddressed are concordance of malleus and incus sizes, bilateral symmetry, whether ossicle size correlates with otitis media, and whether second-branchial arch derivatives have more variability than first-arch derivatives. We sought to quantitatively describe the malleus and incus in a population not heretofore reported, with the following hypotheses in mind: 1) an ear's malleus and incus sizes are concordant; 2) a cranium's malleus and incus sizes have bilateral symmetry; 3) the sizes of the malleus and incus are unrelated to the mastoid-size indicator of childhood otitis media; and 4) second-branchial arch derivatives have more variability than do first-arch derivatives. METHODS: We performed a postmortem material analysis of 41 adult crania without clinical otitis. RESULTS: The sizes of clinically normal mallei (eg, 21.2 to 30.7 mg) and incudes (eg, 24.4 to 37.4 mg) were varied. Concordance of malleus mass and incus mass was found. However, no relation of malleus and incus sizes with mastoid size was found. The variability of first-arch derivatives was similar to that of second-arch derivatives. CONCLUSIONS: Clinically normal mallei and incudes had masses and dimensions that varied even more than previously reported. Nevertheless, bilateral symmetry was exhibited, as was concordance of masses.

Relation of public health staffing to follow-up after newborn hearing screening in three health districts in Georgia, 2009-2015.

OBJECTIVE: To describe the association, or non-association, of public health district staffing (specifically, Early Hearing Detection and Intervention [EHDI] coordinator/navigator) and loss to follow-up in newborns who did not pass hearing screening in selected public health districts in Georgia, USA. METHODS: By Freedom of Information request, data regarding newborn hearing screening and loss to follow-up for diagnostic testing and staffing were acquired for three districts in Georgia for six years. The districts were chosen because their coordinator/navigator positions were unfilled at times. RESULTS: Lapses in staffing of the district EHDI coordinator/navigator position aligned temporally with decreased follow-up. Aggregate three district data showed that follow-up rates in quarter-years with a fulltime navigator were higher than quarter-years without a full-time navigator (p < .001). CONCLUSION: Lapses in staffing dedicated to EHDI navigation-coordination correlated with poorer follow-up after not passing newborn hearing screening.

Comprehensive genetic testing of Chinese SNHL patients and variants interpretation using ACMG guidelines and ethnically matched normal controls.

Hereditary hearing loss is a monogenic disease with high genetic heterogeneity. Variants in more than 100 deafness genes underlie the basis of its pathogenesis. The aim of this study was to assess the ratio of SNVs in known deafness genes contributing to the etiology of both sporadic and familial sensorineural hearing loss patients from China. DNA samples from 1127 individuals, including normal hearing controls (n = 616), sporadic SNHL patients (n = 433), and deaf individuals (n = 78) from 30 hearing loss pedigrees were collected. The NGS tests included analysis of sequence alterations in 129 genes. The variants were interpreted according to the ACMG/AMP guidelines for genetic hearing loss combined with NGS data from 616 ethnically matched normal hearing adult controls. We identified a positive molecular diagnosis in 226 patients with sporadic SNHL (52.19%) and in patients from 17 deafness pedigrees (56.67%). Ethnically matched MAF filtering reduced the variants of unknown significance by 8.7%, from 6216 to 5675. Some complexities that may restrict causative variant identification are discussed. This report highlight the clinical utility of NGS panels identifying disease-causing variants for the diagnosis of hearing loss and underlines the importance of a broad data of control and ACMG/AMP standards for accurate clinical delineation of VUS variants.

The malleus-stapes offset.

BACKGROUND: The malleus-stapes offset create challenges during ossiculoplasty, specifically the positioning of an interposed incus or replacement prosthesis relative to the manubrium. OBJECTIVES: To depict the malleus-stapes offset, its relationship to the orientation of the manubrium as viewed through the external ear canal relative to a horizontal plane, and mastoid pneumatization size. STUDY DESIGN: Postmortem anatomic dissection of 41 bequeathed adult crania (82 temporal bones) without clinical otitis. METHODS: The malleus-stapes offset was analyzed both as a surgeon does intraoperatively and from above through the tegmen tympani. Mastoid sizes were determined radiographically. RESULTS: : From the surgeon's perspective, for the right ear, the "height" (manubrium to caput stapedis) ranged from 0 to 1.5 (mean, 0.5) mm; the "horizontal" offset ranged from 1.0 to 4.0 (mean, 2.2) mm. Differences in angular offset, as viewed transcanally versus trans-tegmenally, averaged 8.5 degrees (range to 53 degrees). Both height and horizontal offset exhibited wide intersubject variability, quite weak bilateral symmetry, and no relationship with either manubrium orientation or mastoid size. CONCLUSION: Reliable determination of the malleus-stapes offset is difficult. Because the error of visual offset assessment exceeds the generally accepted 45 degrees alignment allowance for an ossicular prosthesis, visualized ossiculoplasty alignment should be perfect. The ranges of manubrium-stapes offset do not correlate with either manubrium orientation or the extent of mastoid pneumatization.

Mandibular condylar hypoplasia in children with isolated unilateral congenital aural atresia.

OBJECTIVES/HYPOTHESIS: We hypothesized that children with isolated nonsyndromic unilateral congenital aural atresia have subclinical mandibular condylar hypoplasia ipsilateral to the atretic ear, and that the Jahrsdoerfer score is associated with the degree of condylar hypoplasia. STUDY DESIGN: Retrospective self-controlled case series. METHODS: We reviewed high-resolution computed tomography scans of the temporal bones of 68 children with isolated nonsyndromic unilateral congenital aural atresia. Images were viewed in the transverse (axial) plane perpendicular to the axis of the mandibular ramus and scanned from the mandibular notch to the condylar top. The slice where the condyle had the largest cross-sectional area was measured and checked for correlation with atresia status, age, and sex. The Jahrsdoerfer score of the atretic ear was calculated and correlated with condyle cross-sectional area, age, and sex. RESULTS: Cross-sectional area of the condyle ipsilateral to the atretic ear was, on average, 8.41 mm2 smaller than the contralateral condyle (P < .0001). The Jahrsdoerfer score was not associated with the condylar cross-sectional area, age, or sex. CONCLUSION: Isolated nonsyndromic unilateral congenital aural atresia is associated with mild hypoplasia of the mandibular condyle ipsilateral to the atretic ear. This is consistent with the hypothesis that congenital aural atresia is a variant of craniofacial (hemifacial) microsomia. LEVEL OF EVIDENCE: 4. Laryngoscope, 128:1191-1195, 2018.

Jugular dominance is unrelated to isolated unilateral congenital aural atresia in children.

OBJECTIVES: The right jugular foramen is usually larger than the left in Homo sapiens, but it is unknown if right-sided jugular dominance is also more common amongst those with unilateral congenital aural atresia. We hypothesized that the dominant (i.e. larger) jugular foramen in children with isolated, non-syndromic, unilateral congenital aural atresia would be contralateral to the atretic ear. METHODS: We reviewed high-resolution computed tomography scans of the temporal bones of 70 children with isolated, non-syndromic, unilateral congenital aural atresia. Images were viewed in the transverse (axial) plane by a board-certified neuroradiologist and a board-certified otolaryngologist. Consensus opinion was recorded. Multiple logistic regression was used to assess the impact of age, sex, and side of aural atresia on jugular dominance. RESULTS: Jugular foramen dominance was not associated with the side of aural atresia (P = 0.20), age (P = 0.50) or sex (P = 0.76). Right-sided jugular dominance (46/70, 65.7%) was more common in both left- and right-sided unilateral aural atresia (P = 0.004). CONCLUSIONS: The side of jugular foramen dominance is not associated with the side of aural atresia in children with isolated, non-syndromic, unilateral congenital aural atresia. Right-sided jugular dominance is more common irrespective of unilateral aural atresia.

Cochlear implantation via the middle fossa: surgical and electrode array considerations.

Cochlear implantation via the middle cranial fossa may access the entire length of the cochlea without opening the middle ear. Concerns include safety and electrode array design. The objectives of this study were to determine the depth to the superior portion of the basal turn (SPBT) and distances from the facial and greater petrosal nerves, and to describe some ideas about electrode arrays. The study involved operative dissection of 41 bequeathed otitis-free adult crania (82 temporal bones). Mastoid size was assessed by x-ray. Commercially available MED-EL split arrays were inserted. The depth from the floor of the middle cranial fossa to SPBT of the cochlea ranged from 0.5 mm to 4.2 mm. Small mastoid size correlated with shallow depth. Distances from the centre of the SPBT to the labyrinthine portion of the facial nerve, to the geniculate ganglion, and to the greater petrosal nerve ranged from 1.0 mm to 3.0 mm, 2.0 mm to 3.2 mm, and 1.8 mm to 2.8 mm, respectively. More than 75% of electrodes inserted toward the round window extended into the vestibule. Insertions toward the cochlear apex had a median insertion depth of 12 mm (range 6 mm to 18 mm). The middle cranial fossa approach appears safe and allows electrode access to nearly the full length of the cochlea. Electrode arrays specific for this route of implantation are needed, together with a rigorous study comparing outcomes of this route of implantation with traditional implantation through the facial recess.

Inner ear anomalies in children with isolated unilateral congenital aural atresia.

OBJECTIVES/HYPOTHESIS: We aim to define the frequencies of anomalies of the inner ear, oval window, and round window ipsilateral to isolated non-syndromic unilateral aural atresia. METHODS AND MATERIALS: Retrospective case series. We reviewed high resolution computed tomography scans of the temporal bones of 70 children with isolated non-syndromic unilateral congenital aural atresia. Scans were reviewed according to the Jahrsdoerfer criteria and further evaluated for anomalies of the vestibule, semi-circular canals, cochlea, internal auditory canal and vestibulocochlear nerve. RESULTS: Inner ear dysplasia was seen in two of 70 atretic ears: one with a dysmorphic lateral semicircular canal and another with a large vestibule assimilating the lateral semicircular canal. Abnormalities of the oval window and round window ipsilateral to the atresia were identified in 21% (15) and 7% (5), respectively, of the atretic ears. Oval window and round window abnormalities were associated with disproportionately lower Jahrsdoerfer scores compared to aural atresia patients without these abnormalities (P < 0.001 and P = 0.04, respectively). CONCLUSION: Compared to studies that included syndromic or bilateral atresia cases, we found inner ear and oval window abnormalities less common in children with isolated non-syndromic unilateral aural atresia. However, round window anomalies seem to occur at about the same frequency.

Cilia distribution and polarity in the epithelial lining of the mouse middle ear cavity.

The middle ear conducts sound to the cochlea for hearing. Otitis media (OM) is the most common illness in childhood. Moreover, chronic OM with effusion (COME) is the leading cause of conductive hearing loss. Clinically, COME is highly associated with Primary Ciliary Dyskinesia, implicating significant contributions of cilia dysfunction to COME. The understanding of middle ear cilia properties that are critical to OM susceptibility, however, is limited. Here, we confirmed the presence of a ciliated region near the Eustachian tube orifice at the ventral region of the middle ear cavity, consisting mostly of a lumen layer of multi-ciliated and a layer of Keratin-5-positive basal cells. We also found that the motile cilia are polarized coordinately and display a planar cell polarity. Surprisingly, we also found a region of multi-ciliated cells that line the posterior dorsal pole of the middle ear cavity which was previously thought to contain only non-ciliated cells. Our study provided a more complete understanding of cilia distribution and revealed for the first time coordinated polarity of cilia in the epithelium of the mammalian middle ear, thus illustrating novel structural features that are likely critical for middle ear functions and related to OM susceptibility.

Longitudinal measures of human papillomavirus 6 and 11 viral loads and antibody response in children with recurrent respiratory papillomatosis.

OBJECTIVES: To measure human papillomavirus (HPV) 6 and 11 viral load and antibody response in longitudinal specimens obtained from children with recurrent respiratory papillomatosis and to examine the association of type-specific viral load with clinical severity of disease. DESIGN: Longitudinal pilot study with a median follow-up of 5.4 months. SUBJECTS: The study included 15 children undergoing therapy for recurrent respiratory papillomatosis at the Egleston Children's Hospital, Atlanta, GA, between January 22, 1999, and June 13, 2000. MAIN OUTCOME MEASURES: The kinetics of HPV-6 and HPV-11 viral load and antibody level were examined over time. Longitudinal HPV-6 and HPV-11 viral loads were analyzed for associations with clinical indicators of disease severity. RESULTS: Four children were infected with HPV-11, 4 were infected with HPV-6, and 7 had mixed infections. The HPV-6 and HPV-11 viral loads were stable over time in most of the children. Among children with mixed infections, HPV-6 viral loads were inversely correlated with those of HPV-11 (r = -0.80, P<.001). The HPV-11 infection was significantly associated with more annual surgical procedures (P=.02). Neither HPV-6 nor HPV-11 viral loads were associated with demographic factors or markers of clinical severity. None of the children had detectable antibodies against HPV-6, and only 3 had detectable antibodies against HPV-11 virallike particles. CONCLUSIONS: Our data support the association of HPV-11 infection with clinical severity. Measures of HPV-6 and HPV-11 viral loads are relatively stable over time in most children with recurrent respiratory papillomatosis, suggesting that multiple samples may not be necessary. Cytobrush samples may substitute for tissue biopsy specimens in HPV detection and typing, but not for absolute viral load determination.

Helpful and unhelpful parts of the superior petrosal triangle.

BACKGROUND: The root of zygoma-head of malleus line may guide to the internal auditory canal, but few anatomic studies address its accuracy. HYPOTHESES: The precision of measurements is excellent, the "superior petrosal triangle" components (foramen spinosum, root of zygoma, and head of malleus) have a consistent relationship and accurately guide to Bill's bar, and distances correlate with pneumatization. METHODS: Forty-one bequeathed adult human cranial base specimens (82 temporal bones) were dissected in a surgical manner. RESULTS: Measurements had excellent repeatability. The root of zygoma-head of malleus reference line always projected onto or posterior to Bill's bar, but distances measured had wide ranges. No measurement correlated with pneumatization. CONCLUSION: The root of zygoma-head of malleus line extends onto or posterior to Bill's bar. Measurement guides to the head of the malleus are not predictable.

Universal newborn hearing screening follow-up in two Georgia populations: newborn, mother and system correlates.

BACKGROUND: Nearly half of babies who "fail" their newborn hearing screening do not receive appropriate follow-up. Various explanations have been suggested. OBJECTIVE: To investigate, in two contrasting populations of newborns in Georgia, contemporaneous medical, socio-demographic and screening correlates of follow-up after newborn hearing screening. Three hypotheses were addressed: (1) follow-up correlated with particular medical, social and demographic features; (2) screening performance indicators correlated with follow-up; and (3) screening policies and procedures correlated with follow-up. METHODS: The studied babies, born July 2001 through June 2003 at Atlanta's Piedmont Hospital and Waycross Health District, had failed screening in both ears. Each site had about 4000 births per year, and a plan for outpatient follow-up of in-patient "fail" babies. RESULTS: At Piedmont, where Medicaid funded less than 7% of the births, 57% (130/230) had known follow-up to outpatient screening. In contrast, at Waycross, where Medicaid funded 89.6% of the births, 100% (38/38) had follow-up. The first study hypothesis was only partially supported by the data-from Piedmont, but not Waycross. White race and maternal age 30 years or older correlated with following-up: odds ratios 2.07 (95% CI 1.17, 3.68) and 1.83 (1.05, 3.17), respectively. Private health insurance and marriage trended with following-up. Follow-up rates did not correlate with the rates of screening refusal, missed screening and "fail" rates. Follow-up was unrelated to mothers witnessing the screening, receiving in-person explanations and having appointments arranged for them. The Piedmont and Waycross programs were quite different. Piedmont's began in 2001, mothers typically learned about screening when it was done and "fail" babies were reported to many public health entities. In contrast, the Waycross program was more than 20 years old, prenatal care included teaching about screening and reporting was to one public health entity. CONCLUSIONS: Though maternal socio-demographic features (poor, non-white, young) and access problems have been considered factors for non-follow-up, this study found two program characteristics most important: the lack of prenatal education about newborn hearing screening, and the lack of functionally integrated hospital hearing information with Public Health.

Orientation of the manubrium mallei: inexplicably widely variable.

BACKGROUND: There is no consensus why the manubrium of the malleus, as viewed clinically through the external ear canal, generally points downward and posteriorly. OBJECTIVES: To depict the alignment of the handle of the malleus, viewed clinically through the external auditory canal, relative to the zygomatic arch, the Frankfort plane, and a visual plane proxy and relative to the horizontal semicircular canal. Also, to assess bilateral symmetry and manubrium alignment relative to mastoid pneumatization. STUDY DESIGN: Postmortem anatomic dissection of 41 bequeathed adult crania without clinical otitis. METHODS: The line of the manubrium as viewed through the external ear canal was measured relative to the Frankfort plane, to a proxy of the visual plane, and to the zygomatic arch. Mastoid sizes were determined radiographically. In a subset of 10 crania additionally studied by computed tomography, the manubrium position was checked relative to the horizontal semicircular canal. RESULTS: Relative to the zygomatic arch and Frankfort and visual planes, the range of manubrium angles was at least 45 degrees. Bilateral symmetry was found, each r 0.38 or greater (P < .05). Relative to the horizontal canals, the range of manubrial angles was 30 degrees, with symmetry suggested. Alignment did not correlate with mastoid pneumatization. CONCLUSION: Manubrium orientation as viewed through the external auditory canal is not obviously explicable. The wide variety of manubrium orientations may explain the manubrium-stapes offset that perplexes manubrium-stapes surgical constructions.

Interobserver agreement of coiling of Med-El cochlear implant: plain x-ray studies.

OBJECTIVE: The objective of this study was to describe the interobserver agreement of otologists in judging the extent of cochlear electrode insertion as depicted in plain radiographs done in various projections relative to that of Stenvers (45 degrees from midline). STUDY DESIGN: Ten experienced U.S. MED-EL cochlear implant surgeons, unaware of the image history, judged depths of electrode coiling in 12 images. METHODS: In a bequeathed cranial base specimen, a standard MED-EL Combi 40+ electrode was inserted 3 mm less than fully and stabilized. With a custom cephalostat, the specimen was positioned in the Frankfort horizontal plane. Plain radiographs were made in six projections in 15 degrees increments, from posterior-anterior to near lateral. After the electrode array was withdrawn 5 mm and stabilized, six additional radiographs were similarly taken. RESULTS: Greater variability of assessed coiling was associated with projections other than Stenvers. The coefficient of variation for the Stenvers projection was significantly less than half that of the anterior-posterior and the other projections studied. CONCLUSION: In plain radiographic assessment of the degree of coiling of the MED-EL electrode, interobserver agreement was best for the Stenvers view.

Childhood otitis media and electrically elicited stapedius reflexes in adult cochlear implantees.

BACKGROUND: Electric stapedius reflex thresholds are helpful in programming cochlear implants, but only approximately two-thirds of patients have identifiable reflexes. HYPOTHESIS: Childhood otitis media correlates with absent stapedius reflexes or with high electric stimulation needed to elicit a reflex in cochlear implantees. STUDY POPULATION: Twenty-five adults with acquired hearing loss who underwent implantation with the MED-EL COMBI 40+ standard electrode array. STUDY PARAMETERS: The extent of temporal bone pneumatization, an indicator of childhood otitis media, was measured from preoperative computed tomographic images. Clinical units (in microamperes) needed to elicit a contralateral stapedius reflex, or maximum stimulation tried, were recorded. RESULTS: No definite association of pneumatization volume with intensity of stimulation was observed. The null hypothesis of no association cannot be rejected. CONCLUSION: Childhood otitis media does not seem to explain absent electric stapedius reflexes and the wide range of clinical units needed for maximum comfortable loudness level. Remaining potential explanations probably include the wide range of cochlear neurons that can be electrically stimulated, and that the maximum tolerable stimulation is too low to elicit a stapedius reflex.

Heterotopic gastric mucosa of the oral cavity: a rare entity.

OBJECTIVE: Heterotopic gastric mucosa found within the oral cavity has been reported as a rare occurrence with less than 30 cases in the English literature. We propose that this entity may not be as rare as originally described. METHOD: Five cases of heterotopic gastric mucosa in the oral cavity were collected from three institutions. RESULTS: Five new cases of heterotopic gastric mucosa in the oral cavity were confirmed. CONCLUSIONS: Heterotopic gastric mucosa of the oral cavity may not be as rare as originally thought.

Early cochlear implantation in children allows normal development of central auditory pathways.

The goal of this study was to determine whether there is a sensitive period during early development when a cochlear implantation can occur into a minimally degenerate and/or highly plastic central auditory system. Our measure of central auditory deprivation was latency of the P1 auditory evoked potential, whose generators include auditory thalamocortical areas. Auditory evoked potentials were recorded in 18 congenitally deaf children who were fitted with cochlear implants by 3.5 years of age. The P1 latencies of the children with implants were compared with the P1 latencies of their age-matched peers with normal hearing. There was no significant difference between the P1 latencies of the children with implants and the children with normal hearing. The present results suggest that early implantation occurs in a central auditory system that is minimally degenerate and/or highly plastic. Studies are ongoing to assess the consequences to the developing central auditory system of initiating electrical stimulation at later ages.

Severe diarrhea-dehydration in infancy permanently alters auditory function.

Of the myriad etiologies of sensorineural hearing impairment, metabolic stress is rarely considered. I posit that severe dehydration in conjunction with hypoxia, at least during infancy, prompts permanent changes in the cochlea. In a population-based prospective study of otitis media, children without otitis were found to have at age 4-8 years, worse auditory thresholds if as an infant had been hospitalized for diarrhea-dehydration. What is more, stapedius reflex thresholds tended to be lower in children who had been hospitalized for diarrhea-dehydration: that is, less acoustic energy for arousal or to be frightening. The hypothesis that the transient metabolic stress of dehydration with hypoxia prompts permanent sensorineural hearing impairment with reduced uncomfortable loudness thresholds, is both (1) consistent in an evolutionary sense with a subsequent survival advantage, and (2) subject to verification both by descriptive studies of children undergoing ECMO (ExtraCorporeal Membrane Oxygenation) or care for congenital diaphragmatic hernia, and by animal studies.