Shortened stapedius tendon: a rare cause of conductive hearing loss.

INTRODUCTION: Anomalies of the stapedius tendon have been reported to cause conductive hearing loss; in theory, such anomalies limit the movement of the stapes. OBJECTIVES: To demonstrate a rare cause of conductive hearing loss resulting from anomaly of the stapedius tendon and to compare the clinical findings of this patient to other stapedius tendon anomalies reported in the literature. METHOD: Case report of a single case of shortened stapedius tendon and a review of the English literature on stapedius tendon anomalies. RESULTS: This is a case report of a 15-year-old boy with shortened stapedius tendon causing unilateral hearing loss, accompanied by a review of the literature. Contrary to other reported cases, this patient did not have an ossified tendon, but rather an extremely short tendon. The boy regained normal hearing following excision of the stapedius tendon. CONCLUSION: A shortened stapedius tendon is a very rare diagnosis, yet it should be considered as a possible cause of conductive hearing loss.

Scleraxis is required for differentiation of the stapedius and tensor tympani tendons of the middle ear.

Scleraxis (Scx) is a basic helix-loop-helix transcription factor expressed in tendon and ligament progenitor cells and the differentiated cells within these connective tissues in the axial and appendicular skeleton. Unexpectedly, we found expression of the Scx transgenic reporter mouse, Scx-GFP, in interdental cells, sensory hair cells, and cochlear supporting cells at embryonic day 18.5 (E18.5). We evaluated Scx-null mice to gain insight into the function of Scx in the inner ear. Paradoxical hearing loss was detected in Scx-nulls, with ~50% of the mutants presenting elevated auditory thresholds. However, Scx-null mice have no obvious, gross alterations in cochlear morphology or cellular patterning. Moreover, we show that the elevated auditory thresholds correlate with middle ear infection. Laser interferometric measurement of sound-induced malleal movements in the infected Scx-nulls demonstrates increased impedance of the middle ear that accounts for the hearing loss observed. The vertebrate middle ear transmits vibrations of the tympanic membrane to the cochlea. The tensor tympani and stapedius muscles insert into the malleus and stapes via distinct tendons and mediate the middle ear muscle reflex that in part protects the inner ear from noise-induced damage. Nothing, however, is known about the development and function of these tendons. Scx is expressed in tendon progenitors at E14.5 and differentiated tenocytes of the stapedius and tensor tympani tendons at E16.5-18.5. Scx-nulls have dramatically shorter stapedius and tensor tympani tendons with altered extracellular matrix consistent with abnormal differentiation in which condensed tendon progenitors are inefficiently incorporated into the elongating tendons. Scx-GFP is the first transgenic reporter that identifies middle ear tendon lineages from the time of their formation through complete tendon maturation. Scx-null is the first genetically defined mouse model for abnormal middle ear tendon differentiation. Scx mouse models will facilitate studies of tendon and muscle formation and function in the middle ear.

Development of the stapedius muscle canal and its possible clinical consequences.

OBJECTIVE: To study the development of the stapedius muscle canal in human embryos and foetuses. MATERIALS AND METHODS: 46 temporal bones with ages between 9mm and new-borns were studied. The preparations were dyed using Martins' trichrome technique. RESULTS: Two areas of different embryological origin form the stapedius muscle canal, which contains this muscle and the facial nerve. On the otic capsule, at 11 weeks an extension starts to grow from its caudal part, which moves outwards and near to Reichert's cartilage, forming the footplate and internal wall. The pyramidal eminence comes from the mesenchyme that surrounds the muscle, forming a partition to separate it from the laterohyale portion of Reichert's cartilage. Extensive connections are observed in its development between bone marrow and mesenchyme. At 35 weeks the muscle and nerve start to separate by means of a bony partition. If this partition does not form, there is going to be a dehiscence that could cause peripheral nerve pathology due to the repeated contraction of the muscle, or the dissemination of infections from middle ear. CONCLUSION: During the development of the stapedius muscle canal the presence of dehiscences between the facial nerve and the muscle may have clinical repercussions.

[Morphodifferentiation of the stapedius muscle. Personal contribution]. / Morfodiferenciación del músculo del estribo. Aportación personal.

The aim of this study is to explain the origin of the anomalies and variations that affect the muscle-tendon complex of the stapes. These are interpreted from the model of the stapes muscle differentiation formed by two independent anlagen (Rodríguez-Vázquez, 2005, 2009 and Rodríguez-Vázquez et al., 2010): one for the belly muscle, located on the second branchial arch medially to the facial nerve; and another for the tendon, formed from the internal segment of interhiale. The external segment of the interhiale usually disappears. The pyramidal eminence is organized from the mesenchymal condensation sorrounding the muscle belly, which is independent of the second branchial arch cartilage.

Development of the stapedius muscle and unilateral agenesia of the tendon of the stapedius muscle in a human fetus.

The objective was to analyze the development of the stapedius muscle to understand an isolated unilateral absence of the tendon of the stapedius muscle in a human fetus. The study was made on 50 human embryos and fetuses aged 38 days to 17 weeks post-conception. The stapedius muscle was formed by two anlagen, one for the tendon, which derives from the internal segment of the interhyale and another for the belly, located in the second pharyngeal arch, medially to the facial nerve and near the interhyale. In the interhyale, two segments were observed forming an angle and delimited by the attachment of the belly of the stapedius muscle. The internal segment will form the tendon. The lateral segment of the interhyale was attached to the cranial end of the Reichert's cartilage (laterohyale), and normally it disappears at the beginning of the fetal period. The right unilateral agenesia of the tendon of the stapedius muscle, observed for the first time in a human fetus of 14 weeks post-conception development (PCd), was brought about by the lack of formation or the regression of the internal segment of the interhyale. It presented a belly of the stapedius muscle with an anomalous arrangement, and with a pseudo tendon originated by the persistence of the external segment of the interhyale.

Development of the stapedius muscle and pyramidal eminence in humans.

The aim of the study was to systematize the key developmental phases of the stapedius muscle and the pyramidal eminence to clarify their formation, as well as to understand the variations and anomalies that can affect these structures. Sixty human embryos and fetuses between 38 days and 17 weeks of development were studied. The stapedius muscle is formed by two anlagen, one for the tendon, which derives from the internal segment of the interhyale, and another for the belly, located in the second pharyngeal arch medial to the facial nerve and near the interhyale but forming a completely independent anlage. In the interhyale, two segments were differentiated, these forming an angle; at the vertex, the belly of the stapedius muscle is attached. The internal segment is located from the attachment of the belly of the stapedius muscle to the anlage of the stapes, forming the anlage of the tendon of the stapedius muscle. The external segment completely disappears at the beginning of the fetal period. The pyramidal eminence is formed by an anlage independent of Reichert's cartilage, from the mesenchymal tissue of the tympanic cavity, which condenses around the belly of the stapedius muscle from 12 weeks of post-conception development. The length of the tendon of the stapedius muscle in adults varies, depending on the attachment site of the belly of the stapedius muscle in the interhyale, which would determine the length of the internal segment (anlage of the tendon) and consequently the tendon length. This variation depends on the greater or lesser persistence of the angulation observed during development, between the tendon and the belly of the stapedius muscle.

Contribution to the development of the stapedius muscle structure in human fetuses.

The purpose of this report is to contribute to the knowledge of the morphogenesis of the human stapedius muscle. We have studied the stapedius structure development in 17 temporal bones from human fetuses. Chronological ages were from 48 days (21 mm) to 29 weeks (270 mm). Samples were fixed in 10% formol, decalcified with 2% nitric acid, embedded in Paraplast, sectioned in sequence of 7 microns and were stained with haematoxylin & eosin and Martins' trichrome. Four maturation stages were observed in the development of the stapedius muscle structure. First, the formation of the 'primordium muscularis' in the mesenchyme of the tympanic cavity (promyoblasts) was observed in a 36-mm embryo. Second, its differentiation into myoblasts, and its next cellular fusion to form muscle tubes, was seen in a 99-mm foetus. In the third stage, myofibrogenesis phenomena were developed, to synthesize, the myotube myoblasts, myofilaments, phenomena we observed that took place during the 14th to 17th weeks of development. The last stage was characterized by the presence of striated muscle fibres, at first intermingled with myotubes (18th to 26th weeks), until in the 29th week fetuses (270 mm) the skeletal muscle fibre was completely differentiated. We conclude that in the 29th week of development (270 mm) the stapedius muscle structure is completely differentiated, although the functional development is not complete until birth.

[Origin of the human stapedius muscle]. / Proiskhozhdenie myshtsy stremeni cheloveka.

More than 100 human embryos and fetuses, beginning from 6 mm (25-26 days) up to newborns have been investigated by means of histological serial sections, as well as by means of macro- micropreparation of the tympanic cavity. The m. stapedius is not independent, but takes origin from muscle fasciculi of the musculi digastrii posterior belly. The latter approaches the eminence of the future mastoid process, gives a part of muscle fasciculi into the tympanic cavity; they get through the stilomastoid cleft and run to the hammer neck, forming the m. stapedius. That is why both muscles are innervated from the facial nerve.